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SERONEGATIVE
SPONDYLOARTHROPATHY
PRESENTER:
DR. AMOL GAIKWAD
PG TRAINEE
DEPARTMENT OF ORTHOPAEDICS, GMCH
MODERATOR:
DR. P. GOGOI
ASSISTANT PROFESSOR
DEPARTMENT OF ORTHOPAEDICS, GMCH
INTRODUCTION
 A group of nonrheumatoid disorder in which
striking clinical and familial relationship can be
demonstrated.
SALIENT FEATURES
 Absence of rheumatoid factor.
 Absence of subcutaneous nodule.
 Inflammatory peripheral arthritis .
 Radiological sacroilitis.
SALIENT FEATURES
Evidence of clinical overlap
 Psoriasiform skin or nail lesion
 Ocular inflammation
 Buccal ulceration.
 Genital ulceration.
 Genitourinary infection
 Erythema nodosum.
 Pyoderma gangrenosum.
 Thrombophletitis.
SALIENT FEATURES
 Tendency to familial aggregation.
 Significant association with the HLA B27
antigen.
MEMBERS OF THE GROUP
Association of Spondyloarthropathies
With HLA-B27
Population or Disease Entity HLA-B27 –Positive(%)
Healthy whites 8
Healthy African Americans 4
Ankylosing spondylitis (whites) 92
Ankylosing spondylitis (African
Americans)
50
Reactive arthritis 60-80
Psoriasis associated with spondylitis 60
IBD associated with spondylitis 60
Isolated acute anterior uveitis 50
Undifferentiated spondyloarthropathy 20-25
KEY FEATURES OF SERONEGATIVE
SPONDYLOARTHROPATHIES
 Affect the axial skeleton
 Involvement of sacroiliac joints
 Peripheral joint involvement is usually asymmetrical,
oligoarticular, below waist
 Enthesopathy (pain along tendon insertion sites) is
characteristic
 Usual age < 40 years
 Male preponderance
ANKYLOSING SPONDYLITIS
Ankylos--bent spondylos—vertebrae
 Ankylosing spondylitis (AS) from Greek
 Bechterew's disease,
 Bechterew syndrome,
 Marie Strümpell disease
ANKYLOSING SPONDYLITIS
Chronic, systemic inflammatory
disorder of the axial skeleton.
Sacroiliitis is hallmark of the
disease.
Strong genetic predisposition
(HLA-B27).
HISTORY
 There is evidence from Egyptian and Nubian
skeleton that ankylosing spondylitis existed thousands
of years before Christ
 Barnad Connor (1691)was to give the first
pathological account of the disease .
 Sir Benjamin Brodie in 1850 first gave the
clinical description of the disease. He was the first to
note iritis as a feature of spondylitis.
 In late nineteenth centuries Strumpell , Vladimir
Bechterew and Pierre Marie carefully reported several
cases.
EPIDEMIOLOGY
PREVALENCE:
 0.1-1% of the general population
 Approximately 1-2% of all people who are positive for HLA-B27 develop AS.
 15-20% if they have a first-degree relative with AS.
RACE:
 Higher in Whites and some native Americans when compared to Africans,
Asians and other non white ethnic groups
SEX:
 The male-to-female ratio of AS is 3:1.
AGE:
 usually is from the late teens to age 40 years
CLINICAL FEATURES
 low back ache
 sciatic pain (10%)
 chest pain
 peripheral arthritis(15%)
 pain at the site of tendinous insertion.
 pain and swelling of manubrio-sternal and sterno-clavicular
joints.
SYMPTOMS IN ADVANCED CASES
 The head, neck , entire spine, hips and shoulders all
stiff and fused.
 Spine is so flexed that in some cases vision is
confined to a point just ahead of their foot.
 Pain is characteristically diminished but persist in
some form.
 Mobility is diminished by stiffness rather than pain
and morning stiffness looses its most of painful
component.
 Life is more difficult due to crippling changes but
patient remain relatively uncomplaining.
CHEST & SPINE RIGIDITY
 Spinal movement is restricted in all
direction
 Chest expansion is diminished relatively
early
 Respiration became purely diaphragmatic
 Sacroiliitis as evidence by pain on
stretching of sacroiliac joint.
EXTRA ARTICULAR BONY
TENDERNESS
 symphysis pubis
 greater trochanter
 iliac crest
 ischial tuberosity
 spinous process
 manubrium sterni
 costal cartilage
 heels.
EXTRA-ARTICULAR
MANIFESTATION
 UVEITIS: Most common extraarticular manifestation.
 CARDIOVASCULAR INVOLVEMENT→ aortic insufficiency,
Mitral valve insufficiency, various degrees of atrioventricular
block, including complete heart block.
 NEUROLOGIC INVOLVEMENT→ secondary to fractures of a
fused spine, atlantoaxial subluxation , Cauda equina
syndrome
 PULMONARY INVOLVEMENT→ Restrictive lung disease,
Bilateral apical pulmonary fibrosis .
 RENAL INVOLVEMENT →Amyloidosis , Immunoglobulin A
(IgA) nephropathy
ANKYLOSING SPONDYLITIS IN
WOMEN
 Male to-female ratio of
approximately 3:1.
 More frequent involvement of
cervical spine and symphysis
pubis.
 No significant improvement or
deterioration during pregnancy
JUVENILE ANKYLOSING
SPONDYLITIS
 Onset of symptoms before age 16 years.
 Peripheral arthritis, and Dactylitis are
more common
 Systemic manifestations (eg, fever, weight
loss, anemia, leukocytosis) occur at the
onset of disease
PROGNOSIS
Bad prognostic factors are
 Gross crippling with hip involvement.
 Cardiac anomalies particularly aortic
reflux.
 Fibrotic lung involvement.
 Amyloidosis.
 Severe cases needing high risk
therapy.
CRITERIA FOR DIAGNOSIS
ROME CRITERIA (1961)
 Low back pain and stiffness for >3 months that is not relieved by
rest.
 Pain and stiffness in the thoracic region
 Limited motion in the lumbar spine
 Limited chest expansion
 History or evidence of iritis or its sequelae.
Diagnosis of AS when any clinical criteria present with bilateral
sacroilitis or when 4 of the clinical criteria is present.
CRITERIA FOR DIAGNOSIS
NEW YORK CRITERIA (1984)
 Low back pain with inflammatory characteristics
 Limitation of lumbar spine motion in sagittal and frontal planes
 Decreased chest expansion
 Bilateral sacroiliitis grade 2 or higher
 Unilateral sacroiliitis grade 3 or higher
RADIOLOGICAL FEATURES
 SACROILIITIS:
radiologically it is graded as,
 Grade 0:Unequivocally normal.
 Grade 1: Possibly abnormal.
 Grade 2: Definite marginal sclerosis.
 Grade 3: definite erosion and sclerosis.
 Grade 4: complete obliteration
TESTS FOR SACROILIITIS
 GAENSLEN'S TEST:
the hip joint is flexed maximally on one
side and the opposite hip joint is extended, stressing
both sacroiliac joints simultaneously.
 SACRAL THRUST TEST:
With the patient prone, the examiner
applies an anteriorly directed pressure over the
sacrum.
TESTS FOR SACROILIITIS
 The (Patrick’s) FABER Test:
The patient is positioned in supine. The
leg is placed in a figure-4 position (hip flexed and
abducted with the lateral ankle (malleolus) resting
on the contralateral thigh just above (/proximal to)
the knee).
SCHOBER’S TEST
 Test for lumbar flexion.
 While the patient is in a standing position the examiner makes
a mark approximately at the level of L5 (fifth lumbar vertebra).
 Two points are marked: 5 cm below and 10 cm above this point
(for a total of 15 cm distance).
 Then the patient is asked to touch his/her toes while keeping
the knees straight.
 If the distance of the two points do not increase by at least 5 cm
(with the total distance greater than 20 cm), then this is a sign
of restriction in the lumbar flexion.
SPINAL CHANGES
 Syndesmophyte
 Bambooing of spine
 Romanus lesions
SPINAL CHANGES
 Squaring of vertebrae.
 Osteoporosis is a late feature
 Spondylodiscitis
SPINAL CHANGES
 Rail road track line
 Daggers sign
OTHER CHANGES
 Peripheral joints→ fewer tendencies for joint
erosion and greater tendency for bony ankylosis.
 Erosion ,sclerosis, ankylosis of cartilaginous joints
 Ossification of ligaments.
 Irregular periosteal new bone formation at various
site
 Erosion of posterior surface of calcaneum, greater
tuberosity of humerus, greater trochanter.
 Spur formation at external occipital protuberance,
insertion of planter fascia.
LABORATORY FINDINGS
No laboratory tests are specific for ankylosing spondylitis (AS).
 Negative rheumatoid factor.
 a normochromic normocytic anemia of chronic disease.
 Include raised level of alpha 2 and gamma globulin.
 Raised ESR or C-reactive protein.
 Elevated Alkaline phosphatase.
 Slight elevation of cerebrospinal fluid protein.
 Increase complements level .
 Features of altered immunity.
 HLA-B27 positivity is present in 90%.
LABORATORY STUDIES
Antinuclear antibodies in ankylosing spondylitis,
psoriatic arthritis, and psoriasis.
Granulocyte-specific (GS) antibody, which reacts
with nuclei of mature granulocytes which were found to be
positive in some patients with ankylosing spondylitis,
psoriatic arthritis, and psoriasis.
DIFFERENTIAL DIAGNOSIS
Intervertebral and paravertebral ossification→
 Reiter’s spondylitis
 Psoriatic arthritis
 Myositis ossificans
 Senile ankylosing hyperostosis.( forestier’s disease.)
Spinal stiffening opacification of intervertebral joints→
 Onchronosis
 Chondrocalcinosis
 Haemochromatosis
 Hyperparathyroidism
 Acromegaly
MANAGEMENT
Physical therapy
 Breathing exercise.
 Isometric muscle exercise to strengthen quadriceps, glutei,
abdominal muscles
 Prone lying to prevent hip contracture.
 Neck movement and shoulder shrugging and arms exercise,
 Postural or back discipline.
 Water therapy and swimming are excellent activities to maintain
mobility and fitness.
MANAGEMENT
Non operative deformity correction→
– Spinal brace →usually reserved for
deformity not correctable by exercise.
– Divided plaster bed, halo splint and for
severe deformity.
Measure to improve visual field→
– Prismatic spectacle for patient with gross
kyphosis.
– Adjustable mirror for attachment to bed to
provide patient with improve view of
surrounding.
MEDICAL THERAPY
 Nonsteroidal anti-inflammatory drugs
 Sulfasalazine (Adult Dose 2000-3000 mg/d PO divided bid/tid )
 Methotrexate (reports regarding the efficacy of this agent in AS
conflict.)
 The TNF-a inhibitors:
Etanercept(25 mg SC 2 times/wk or 50 mg SC qwk) and
Infliximab (5 mg/kg IV at weeks 0, 2, and 6, then q4-8wk
depending on clinical response; dose may be increased to 10
mg/kg/dose if needed) are approved therapies
Adalimumab (40 mg SC every other week, )has shown
promise in clinical trials to date.
MEDICAL THERAPY
CORTICOSTEROIDS →
 Oral corticosteroids occasionally are helpful in
controlling symptoms; only for short-term
management.
 Local corticosteroid injections are useful for
symptomatic sacroiliitis peripheral enthesitis, and
arthritis
SURGICAL TREATMENT
OSTEOTOMY OF LUMBAR SPINE→
 To improve vision.
 Relief G.I. symptom.
 Improve appearance.
SURGICAL TREATMENT
 SMITH-PETERSEN OSTEOTOMY:
excellent option for correction of smaller
degrees of spinal deformity.
bone is removed through the pars and facet
joints.
 PEDICLE SUBTRACTION
OSTEOTOMY/THOMASEN OSTEOTOMY
resection of posterior wedge and resection
of pedicles
 EGGSHELL OSTEOTOMY
spinal shortening procedure with anterior
decancellization and removal of posterior
elements, instrumentation, deformity
correction and fusion.
SURGICAL TREATMENT
OSTEOTOMY OF CERVICAL SPINE
 to elevate the chin from the sternum.
 to prevent atlantoaxial and cervical
subluxation and dislocations.
 to relieve tracheal and esophageal distortion.
 to prevent irritation of the spinal cord tracts
or excessive traction on the nerve roots.
OSTEOTOMY OF CERVICAL SPINE
 SIMMONS OSTEOTOMY:
here the spinous process and laminae of C7 together
with the inferior portion of C6 and the upper portion of Tl is
removed.
 FREEMAN PROCEDURE:
this is performed when there is minimal passive
cervical motion as demonstrated by skeletal traction but
patient cannot elevate his chin. Neck is gradually extended
with a halo. When some of cervical lordosis is achieved , an
arthrodesis from T3 to occiput is carried out with the halo still
in place.
ATLANTOAXIAL INSTABILITY
 Posterior arthrodesis of Cl-C2:
Surgical stabilization is justified when
there is gross symptomatic atlantoaxial
instability and the patient is at risk. Stabilization
can be effectively achieved by a Gallie-type
posterior atlantoaxial arthrodesis.
 Transoral decompression:
In the few instances when the
odontoid is causing major anterior pressure that
cannot be reduced, Transoral resection of the
odontoid may be required.
PSORIATIC ARTHRITIS
 Psoriatic Arthritis is a chronic disease
characterized by inflammation of the
skin (psoriasis) and joints (arthritis)
 Psoriasis causes a scaly skin rash on
the elbows, knees and scalp and
swelling and pain in joints
 Usually affects the wrists, knees,
ankles, fingers and toes. It can also
affect the back.
EPIDEMIOLOGY
 Prevalence 5-7% of the population with psoriasis
 Race more common in whites
 Sex: The male-to-female ratio is 1:1
 Age: Age of onset is usually 30-55 years.
Juvenile form, the onset is at 9-11 years.
ETIOLOGY
GENETIC PREDISPOSITION →this theory is supported by
 a strong family history.
 a 70% concordance between monozygotic twins
ENVIRONMENTAL THEORY
 some evidence suggests that infectious agents (eg, streptococci, staphylococci)
may have a place in pathogenesis of psoriasis,
 Involves a process of super antigens reacting with auto antigens.
IMMUNOLOGICAL THEORY
 Evidence suggests that T-cells play an active role in PsA, as they are present even
early on in psoriatic lesions.
 The theory of immunological involvement also is supported by the fact that
immunosuppressive agents are successful in treating PsA.
CLINICAL FEATURES
 Psoriasis precedes arthritis in 60-80% of the
patients.
 Duration between onset of psoriasis and arthritis
usually is less than 10 years.
 Insidious in onset, but acute onset has been
reported in one third of all patients.
 Symptoms consist of joint pains, as well as morning
stiffness and onychodystrophy (ie, onycholysis,
pitting of the nails).
 One third of patients may develop inflammatory
ocular symptoms.
 Elderly onset (>60 y) psoriatic arthritis has more
severe onset and more destructive outcome than in
younger subjects.
VARIETY OF FORMS
 Asymmetric oligoarthritis or monoarthritis
 Symmetric polyarthritis
 Arthritis mutilans
 Juvenile psoriatic arthritis
 Spondylitis
PSORIATIC SPONDYLITIS
 The first type is characterized by involvement of the axial spine
alone.
 Radiologic evidence shows sacroiliitis, as well as nonmarginal
and asymmetrical syndesmophytes.
 The lumbar spine is the most commonly affected area.
 Enthesopathic erosions also may be observed, often at the
insertion of the Achilles tendon into the calcaneus.
 The second type of spondylitis is characterized by an overlap of
involvement of the spinal and peripheral joints.
LABORATORY STUDIES
 No laboratory studies can confirm the presence of PsA.
 Nonspecific elevation in erythrocyte sedimentation rate.
 Serum uric acid elevations have been observed in 10-20% of the
patients,
 Raised level of circulating immunocomplexes.
 Synovial fluid analysis typically reveals inflammatory arthritis.
 Raised C reactive protein and fibrinogen which correlate with
disease activity.
IMAGING STUDIES
 Lack of porosis despite destructive changes.
 Sparing of metacarpophalangeal joint.
 Involvement of radioulnar joint rather than
radionavicular joint at wrist.
 Involvement of distal interphalangeal joint
of fingers and toes with marginal erosion.
 Erosion of tips of terminal phalanx.( most
clearly in hallux.)
IMAGING STUDIES
 Pencil-in-cup deformity
 Joint space narrowing in the
interphalangeal (IP) joints, possibly with
ankylosis
 Unilateral or symmetric sacroiliitis
 Large, nonmarginal, unilateral,
asymmetric syndesmophytes
(intervertebral bony bridges) may be
present in cervical, thoracic, and lumbar
spine, often sparing some of the segments.
MANAGEMENT
Medical therapy
 Nonsteroidal anti-inflammatory drugs (NSAIDs)→.
Meclofenamate (Meclomen) is most commonly used. (50 mg
q4-6h, not to exceed 400 mg/d)
 Methotrexate -- Ameliorates symptoms of inflammation
 Adalimumab (Humira) -- Recombinant human IgG1
monoclonal antibody specific for human TNF.
MEDICAL THERAPY
 Infliximab (Remicade) -- Neutralizes cytokine TNF-
alpha and inhibits its binding to TNF-alpha receptor.
 Cyclosporine (Sandimmune, Neoral) -- Symptomatic
improvement in both skin and joint manifestations
usually is seen in 2-8 wk
PHYSICAL THERAPY
 Rest, splintage, HOT and cold modalities to reduce
pain and spasm in acute stage.
 Gradual range of motion, muscle strengthening
exercise and stretching in sub acute and chronic stage.
SURGICAL MANAGEMENT
 Surgical Treatment should be aimed at pain relief or increasing
the patient's function. This include
 Hip and knee joint replacements.
 Arthrodesis or arthroplasty for joints such as the thumb PIP.
 Release of joint contracture.
 In arthritis mutilans, surgical intervention usually is directed
toward salvage of the hand. Combinations of arthrodesis,
arthroplasty, and bone grafts to lengthen the digits may be used.
 The goal is to maintain the pinch mechanism of the thumb and
the first 2 fingers.
REACTIVE ARTHRITIS (REITER’S
SYNDROME)
 Aseptic inflammatory polyarthritis that usually follows
nongonococcal urethritis or infectious dysentery
 The classic triad
nonspecific urethritis,
arthritis
conjunctivitis.
EPIDEMIOLOGY
 The incidence is about 30-40 cases per 100,000 adults
 Enteric form affect males and females with the same
frequency.
 Venereally acquired form occurs in a male-to-female
ratio of 9:1.
 Most patients are aged 20-40 years
PATHOPHYSIOLOGY
 Usually triggered by a genitourinary or gastrointestinal
infection.
 Chlamydia trachomatis has been implicated.
 The mechanism of the interaction of the inciting
organism with the host (often HLA-B27 positive)
leading to the development of reactive arthritis is not
known.
 Synovial fluid cultures are negative for enteric
organisms or Chlamydia species.
LABORATORY STUDIES
 Elevated markedly acute phase reactants, including (ESR) and
(CRP),during acute phase
 Normocytic normochromic anemia
 IgA antibodies to specific bacterial antigens
 aseptic pyuria.
 Synovial fluid analysis reveals a high white blood cell count,
most often with elevated polymorphonuclear leukocytes acutely.
Gram stain and culture results are negative
LABORATORY STUDIES
 Throat, stool, or urogenital tract cultures and serologic
techniques for the detection of Chlamydia species,
including PCR, could be considered.
 HLA-B27 testing yields positive results in 65-96% of
cases
IMAGING STUDIES
 Early in the disease, no abnormalities are found on radiograph.
 In more advanced or long-term disease, periosteal reaction and
proliferation at sites of tendon insertion can be observed.
 Exuberant plantar spurs.
 Sacroiliitis occurs in less than 10% of acute cases but may be
observed in 50% of those patients with chronic severe disease.
 Syndesmophytes are usually asymmetrical.
TREATMENT
 Nonsteroidal anti-inflammatory drug, such as
indomethacin (Indocin).
 Evidence of erosion or sacroiliitis, methotrexate and
azathioprine have been shown to be helpful.
 Doxycycline or an analog for up to three months in
Chlamydia-induced reactive arthritis.
TREATMENT
 Disease-modifying antirheumatic drugs→ In patients
with persistent symptoms, sulfasalazine in dosages of 1
to 3 g daily, have been useful
 Intra-articular steroid injections in patients with large
knee effusions can be helpful.
ENTEROPATHIC ARTHRITIS
 The association of arthritis with inflammatory gut
disease has been termed as enteropathic arthritis. This
type of arthritis is common in crohn’s disease,
ulcerarative colitis, Whipple’s disease.
 Incidence of enteropathic synovitis in Crohn’s disease
is 20.7%, 11% in ulcerative colitis, and 90% of pt. with
Whipple’s disease.
CLINICAL FEATURES
 Recurrent attack of acute synovitis monoarticular in most cases
 Knee is the most commonly affected
 Erythema nodosum
 In Whipple’s disease Arthritis is rather variable
 Pseudospondylitis characterized by absence of sacroiliitis and
response to antibiotic therapy reported in association with Whipple’s
disease.
 Tendency of exacerbation of gut and joint disease together
 Surgery abolishes arthritis in ulcerative colitis not in Crohn disease
 About 16% patient shows radiological sacroiliitis, 6% have
unequivocal sign of the disease.
INVESTIGATION
 No characteristic finding ,only excludes other condition.
 In Whipple’s disease diagnosis is confirmed by PAS.
 Positive macrophage in small intestinal biopsy specimen
TREATMENT
 NSAID in short course to control joint disease.
 Aspiration of swollen joint and instillation of hydrocortisone
produces rapid relief.
 Gut disease is treated accordingly and should not be influenced
by joint disease..
 In ulcerative colitis Best therapy lies in control of gut disease.
 Maintenance of spinal and thoracic mobility by regular exercise
program.
 Whipples disease usually response to prolonged antibiotic
treatment usually with tetracycline. Arthritis abates as the gut
disease is controlled. In persistence cases antibiotic regime
should be revised.
BEHCET’S SYNDROME
 Is a multisystem disorder presenting with recurrent
oral and genital ulcerations as well as ocular involvement.
 Internationally agreed diagnostic criteria are:-
Recurrent oral ulceration plus any of the following two→
 recurrent genital ulceration
 eye lesion
 skin lesion
 pathergy test
Arthritis of Behcet’s is not deforming and affect knees and ankle.
CONCLUSION
Despite active interest in spondyloarthritis in recent
years there remains many unresolved issues. For
instance, should the ultimate criteria –‘the index of
truth’ continue to be radiological sacroiliitis or should
it be presence of HLA B27. The answer to this could
derived from a more global approach along the
following line→
– Continued recognition of importance of characteristics
clinical feature.
– Continued recognition of importance of radiological
sacroiliitis.
– Continued recognition of importance of HLA B27.
– More flexible approach to allow wide spectrums of disease to
be included in this group.
THANK
YOU!!

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Seronegative spondyloarthropathy

  • 1. SERONEGATIVE SPONDYLOARTHROPATHY PRESENTER: DR. AMOL GAIKWAD PG TRAINEE DEPARTMENT OF ORTHOPAEDICS, GMCH MODERATOR: DR. P. GOGOI ASSISTANT PROFESSOR DEPARTMENT OF ORTHOPAEDICS, GMCH
  • 2. INTRODUCTION  A group of nonrheumatoid disorder in which striking clinical and familial relationship can be demonstrated.
  • 3. SALIENT FEATURES  Absence of rheumatoid factor.  Absence of subcutaneous nodule.  Inflammatory peripheral arthritis .  Radiological sacroilitis.
  • 4. SALIENT FEATURES Evidence of clinical overlap  Psoriasiform skin or nail lesion  Ocular inflammation  Buccal ulceration.  Genital ulceration.  Genitourinary infection  Erythema nodosum.  Pyoderma gangrenosum.  Thrombophletitis.
  • 5. SALIENT FEATURES  Tendency to familial aggregation.  Significant association with the HLA B27 antigen.
  • 7. Association of Spondyloarthropathies With HLA-B27 Population or Disease Entity HLA-B27 –Positive(%) Healthy whites 8 Healthy African Americans 4 Ankylosing spondylitis (whites) 92 Ankylosing spondylitis (African Americans) 50 Reactive arthritis 60-80 Psoriasis associated with spondylitis 60 IBD associated with spondylitis 60 Isolated acute anterior uveitis 50 Undifferentiated spondyloarthropathy 20-25
  • 8. KEY FEATURES OF SERONEGATIVE SPONDYLOARTHROPATHIES  Affect the axial skeleton  Involvement of sacroiliac joints  Peripheral joint involvement is usually asymmetrical, oligoarticular, below waist  Enthesopathy (pain along tendon insertion sites) is characteristic  Usual age < 40 years  Male preponderance
  • 9. ANKYLOSING SPONDYLITIS Ankylos--bent spondylos—vertebrae  Ankylosing spondylitis (AS) from Greek  Bechterew's disease,  Bechterew syndrome,  Marie Strümpell disease
  • 10. ANKYLOSING SPONDYLITIS Chronic, systemic inflammatory disorder of the axial skeleton. Sacroiliitis is hallmark of the disease. Strong genetic predisposition (HLA-B27).
  • 11. HISTORY  There is evidence from Egyptian and Nubian skeleton that ankylosing spondylitis existed thousands of years before Christ  Barnad Connor (1691)was to give the first pathological account of the disease .  Sir Benjamin Brodie in 1850 first gave the clinical description of the disease. He was the first to note iritis as a feature of spondylitis.  In late nineteenth centuries Strumpell , Vladimir Bechterew and Pierre Marie carefully reported several cases.
  • 12. EPIDEMIOLOGY PREVALENCE:  0.1-1% of the general population  Approximately 1-2% of all people who are positive for HLA-B27 develop AS.  15-20% if they have a first-degree relative with AS. RACE:  Higher in Whites and some native Americans when compared to Africans, Asians and other non white ethnic groups SEX:  The male-to-female ratio of AS is 3:1. AGE:  usually is from the late teens to age 40 years
  • 13. CLINICAL FEATURES  low back ache  sciatic pain (10%)  chest pain  peripheral arthritis(15%)  pain at the site of tendinous insertion.  pain and swelling of manubrio-sternal and sterno-clavicular joints.
  • 14. SYMPTOMS IN ADVANCED CASES  The head, neck , entire spine, hips and shoulders all stiff and fused.  Spine is so flexed that in some cases vision is confined to a point just ahead of their foot.  Pain is characteristically diminished but persist in some form.  Mobility is diminished by stiffness rather than pain and morning stiffness looses its most of painful component.  Life is more difficult due to crippling changes but patient remain relatively uncomplaining.
  • 15. CHEST & SPINE RIGIDITY  Spinal movement is restricted in all direction  Chest expansion is diminished relatively early  Respiration became purely diaphragmatic  Sacroiliitis as evidence by pain on stretching of sacroiliac joint.
  • 16. EXTRA ARTICULAR BONY TENDERNESS  symphysis pubis  greater trochanter  iliac crest  ischial tuberosity  spinous process  manubrium sterni  costal cartilage  heels.
  • 17. EXTRA-ARTICULAR MANIFESTATION  UVEITIS: Most common extraarticular manifestation.  CARDIOVASCULAR INVOLVEMENT→ aortic insufficiency, Mitral valve insufficiency, various degrees of atrioventricular block, including complete heart block.  NEUROLOGIC INVOLVEMENT→ secondary to fractures of a fused spine, atlantoaxial subluxation , Cauda equina syndrome  PULMONARY INVOLVEMENT→ Restrictive lung disease, Bilateral apical pulmonary fibrosis .  RENAL INVOLVEMENT →Amyloidosis , Immunoglobulin A (IgA) nephropathy
  • 18. ANKYLOSING SPONDYLITIS IN WOMEN  Male to-female ratio of approximately 3:1.  More frequent involvement of cervical spine and symphysis pubis.  No significant improvement or deterioration during pregnancy
  • 19. JUVENILE ANKYLOSING SPONDYLITIS  Onset of symptoms before age 16 years.  Peripheral arthritis, and Dactylitis are more common  Systemic manifestations (eg, fever, weight loss, anemia, leukocytosis) occur at the onset of disease
  • 20. PROGNOSIS Bad prognostic factors are  Gross crippling with hip involvement.  Cardiac anomalies particularly aortic reflux.  Fibrotic lung involvement.  Amyloidosis.  Severe cases needing high risk therapy.
  • 21. CRITERIA FOR DIAGNOSIS ROME CRITERIA (1961)  Low back pain and stiffness for >3 months that is not relieved by rest.  Pain and stiffness in the thoracic region  Limited motion in the lumbar spine  Limited chest expansion  History or evidence of iritis or its sequelae. Diagnosis of AS when any clinical criteria present with bilateral sacroilitis or when 4 of the clinical criteria is present.
  • 22. CRITERIA FOR DIAGNOSIS NEW YORK CRITERIA (1984)  Low back pain with inflammatory characteristics  Limitation of lumbar spine motion in sagittal and frontal planes  Decreased chest expansion  Bilateral sacroiliitis grade 2 or higher  Unilateral sacroiliitis grade 3 or higher
  • 23. RADIOLOGICAL FEATURES  SACROILIITIS: radiologically it is graded as,  Grade 0:Unequivocally normal.  Grade 1: Possibly abnormal.  Grade 2: Definite marginal sclerosis.  Grade 3: definite erosion and sclerosis.  Grade 4: complete obliteration
  • 24. TESTS FOR SACROILIITIS  GAENSLEN'S TEST: the hip joint is flexed maximally on one side and the opposite hip joint is extended, stressing both sacroiliac joints simultaneously.  SACRAL THRUST TEST: With the patient prone, the examiner applies an anteriorly directed pressure over the sacrum.
  • 25. TESTS FOR SACROILIITIS  The (Patrick’s) FABER Test: The patient is positioned in supine. The leg is placed in a figure-4 position (hip flexed and abducted with the lateral ankle (malleolus) resting on the contralateral thigh just above (/proximal to) the knee).
  • 26. SCHOBER’S TEST  Test for lumbar flexion.  While the patient is in a standing position the examiner makes a mark approximately at the level of L5 (fifth lumbar vertebra).  Two points are marked: 5 cm below and 10 cm above this point (for a total of 15 cm distance).  Then the patient is asked to touch his/her toes while keeping the knees straight.  If the distance of the two points do not increase by at least 5 cm (with the total distance greater than 20 cm), then this is a sign of restriction in the lumbar flexion.
  • 27. SPINAL CHANGES  Syndesmophyte  Bambooing of spine  Romanus lesions
  • 28. SPINAL CHANGES  Squaring of vertebrae.  Osteoporosis is a late feature  Spondylodiscitis
  • 29. SPINAL CHANGES  Rail road track line  Daggers sign
  • 30. OTHER CHANGES  Peripheral joints→ fewer tendencies for joint erosion and greater tendency for bony ankylosis.  Erosion ,sclerosis, ankylosis of cartilaginous joints  Ossification of ligaments.  Irregular periosteal new bone formation at various site  Erosion of posterior surface of calcaneum, greater tuberosity of humerus, greater trochanter.  Spur formation at external occipital protuberance, insertion of planter fascia.
  • 31. LABORATORY FINDINGS No laboratory tests are specific for ankylosing spondylitis (AS).  Negative rheumatoid factor.  a normochromic normocytic anemia of chronic disease.  Include raised level of alpha 2 and gamma globulin.  Raised ESR or C-reactive protein.  Elevated Alkaline phosphatase.  Slight elevation of cerebrospinal fluid protein.  Increase complements level .  Features of altered immunity.  HLA-B27 positivity is present in 90%.
  • 32. LABORATORY STUDIES Antinuclear antibodies in ankylosing spondylitis, psoriatic arthritis, and psoriasis. Granulocyte-specific (GS) antibody, which reacts with nuclei of mature granulocytes which were found to be positive in some patients with ankylosing spondylitis, psoriatic arthritis, and psoriasis.
  • 33. DIFFERENTIAL DIAGNOSIS Intervertebral and paravertebral ossification→  Reiter’s spondylitis  Psoriatic arthritis  Myositis ossificans  Senile ankylosing hyperostosis.( forestier’s disease.) Spinal stiffening opacification of intervertebral joints→  Onchronosis  Chondrocalcinosis  Haemochromatosis  Hyperparathyroidism  Acromegaly
  • 34. MANAGEMENT Physical therapy  Breathing exercise.  Isometric muscle exercise to strengthen quadriceps, glutei, abdominal muscles  Prone lying to prevent hip contracture.  Neck movement and shoulder shrugging and arms exercise,  Postural or back discipline.  Water therapy and swimming are excellent activities to maintain mobility and fitness.
  • 35. MANAGEMENT Non operative deformity correction→ – Spinal brace →usually reserved for deformity not correctable by exercise. – Divided plaster bed, halo splint and for severe deformity. Measure to improve visual field→ – Prismatic spectacle for patient with gross kyphosis. – Adjustable mirror for attachment to bed to provide patient with improve view of surrounding.
  • 36. MEDICAL THERAPY  Nonsteroidal anti-inflammatory drugs  Sulfasalazine (Adult Dose 2000-3000 mg/d PO divided bid/tid )  Methotrexate (reports regarding the efficacy of this agent in AS conflict.)  The TNF-a inhibitors: Etanercept(25 mg SC 2 times/wk or 50 mg SC qwk) and Infliximab (5 mg/kg IV at weeks 0, 2, and 6, then q4-8wk depending on clinical response; dose may be increased to 10 mg/kg/dose if needed) are approved therapies Adalimumab (40 mg SC every other week, )has shown promise in clinical trials to date.
  • 37. MEDICAL THERAPY CORTICOSTEROIDS →  Oral corticosteroids occasionally are helpful in controlling symptoms; only for short-term management.  Local corticosteroid injections are useful for symptomatic sacroiliitis peripheral enthesitis, and arthritis
  • 38. SURGICAL TREATMENT OSTEOTOMY OF LUMBAR SPINE→  To improve vision.  Relief G.I. symptom.  Improve appearance.
  • 39. SURGICAL TREATMENT  SMITH-PETERSEN OSTEOTOMY: excellent option for correction of smaller degrees of spinal deformity. bone is removed through the pars and facet joints.  PEDICLE SUBTRACTION OSTEOTOMY/THOMASEN OSTEOTOMY resection of posterior wedge and resection of pedicles  EGGSHELL OSTEOTOMY spinal shortening procedure with anterior decancellization and removal of posterior elements, instrumentation, deformity correction and fusion.
  • 40. SURGICAL TREATMENT OSTEOTOMY OF CERVICAL SPINE  to elevate the chin from the sternum.  to prevent atlantoaxial and cervical subluxation and dislocations.  to relieve tracheal and esophageal distortion.  to prevent irritation of the spinal cord tracts or excessive traction on the nerve roots.
  • 41. OSTEOTOMY OF CERVICAL SPINE  SIMMONS OSTEOTOMY: here the spinous process and laminae of C7 together with the inferior portion of C6 and the upper portion of Tl is removed.  FREEMAN PROCEDURE: this is performed when there is minimal passive cervical motion as demonstrated by skeletal traction but patient cannot elevate his chin. Neck is gradually extended with a halo. When some of cervical lordosis is achieved , an arthrodesis from T3 to occiput is carried out with the halo still in place.
  • 42. ATLANTOAXIAL INSTABILITY  Posterior arthrodesis of Cl-C2: Surgical stabilization is justified when there is gross symptomatic atlantoaxial instability and the patient is at risk. Stabilization can be effectively achieved by a Gallie-type posterior atlantoaxial arthrodesis.  Transoral decompression: In the few instances when the odontoid is causing major anterior pressure that cannot be reduced, Transoral resection of the odontoid may be required.
  • 43. PSORIATIC ARTHRITIS  Psoriatic Arthritis is a chronic disease characterized by inflammation of the skin (psoriasis) and joints (arthritis)  Psoriasis causes a scaly skin rash on the elbows, knees and scalp and swelling and pain in joints  Usually affects the wrists, knees, ankles, fingers and toes. It can also affect the back.
  • 44. EPIDEMIOLOGY  Prevalence 5-7% of the population with psoriasis  Race more common in whites  Sex: The male-to-female ratio is 1:1  Age: Age of onset is usually 30-55 years. Juvenile form, the onset is at 9-11 years.
  • 45. ETIOLOGY GENETIC PREDISPOSITION →this theory is supported by  a strong family history.  a 70% concordance between monozygotic twins ENVIRONMENTAL THEORY  some evidence suggests that infectious agents (eg, streptococci, staphylococci) may have a place in pathogenesis of psoriasis,  Involves a process of super antigens reacting with auto antigens. IMMUNOLOGICAL THEORY  Evidence suggests that T-cells play an active role in PsA, as they are present even early on in psoriatic lesions.  The theory of immunological involvement also is supported by the fact that immunosuppressive agents are successful in treating PsA.
  • 46. CLINICAL FEATURES  Psoriasis precedes arthritis in 60-80% of the patients.  Duration between onset of psoriasis and arthritis usually is less than 10 years.  Insidious in onset, but acute onset has been reported in one third of all patients.  Symptoms consist of joint pains, as well as morning stiffness and onychodystrophy (ie, onycholysis, pitting of the nails).  One third of patients may develop inflammatory ocular symptoms.  Elderly onset (>60 y) psoriatic arthritis has more severe onset and more destructive outcome than in younger subjects.
  • 47. VARIETY OF FORMS  Asymmetric oligoarthritis or monoarthritis  Symmetric polyarthritis  Arthritis mutilans  Juvenile psoriatic arthritis  Spondylitis
  • 48. PSORIATIC SPONDYLITIS  The first type is characterized by involvement of the axial spine alone.  Radiologic evidence shows sacroiliitis, as well as nonmarginal and asymmetrical syndesmophytes.  The lumbar spine is the most commonly affected area.  Enthesopathic erosions also may be observed, often at the insertion of the Achilles tendon into the calcaneus.  The second type of spondylitis is characterized by an overlap of involvement of the spinal and peripheral joints.
  • 49. LABORATORY STUDIES  No laboratory studies can confirm the presence of PsA.  Nonspecific elevation in erythrocyte sedimentation rate.  Serum uric acid elevations have been observed in 10-20% of the patients,  Raised level of circulating immunocomplexes.  Synovial fluid analysis typically reveals inflammatory arthritis.  Raised C reactive protein and fibrinogen which correlate with disease activity.
  • 50. IMAGING STUDIES  Lack of porosis despite destructive changes.  Sparing of metacarpophalangeal joint.  Involvement of radioulnar joint rather than radionavicular joint at wrist.  Involvement of distal interphalangeal joint of fingers and toes with marginal erosion.  Erosion of tips of terminal phalanx.( most clearly in hallux.)
  • 51. IMAGING STUDIES  Pencil-in-cup deformity  Joint space narrowing in the interphalangeal (IP) joints, possibly with ankylosis  Unilateral or symmetric sacroiliitis  Large, nonmarginal, unilateral, asymmetric syndesmophytes (intervertebral bony bridges) may be present in cervical, thoracic, and lumbar spine, often sparing some of the segments.
  • 52. MANAGEMENT Medical therapy  Nonsteroidal anti-inflammatory drugs (NSAIDs)→. Meclofenamate (Meclomen) is most commonly used. (50 mg q4-6h, not to exceed 400 mg/d)  Methotrexate -- Ameliorates symptoms of inflammation  Adalimumab (Humira) -- Recombinant human IgG1 monoclonal antibody specific for human TNF.
  • 53. MEDICAL THERAPY  Infliximab (Remicade) -- Neutralizes cytokine TNF- alpha and inhibits its binding to TNF-alpha receptor.  Cyclosporine (Sandimmune, Neoral) -- Symptomatic improvement in both skin and joint manifestations usually is seen in 2-8 wk
  • 54. PHYSICAL THERAPY  Rest, splintage, HOT and cold modalities to reduce pain and spasm in acute stage.  Gradual range of motion, muscle strengthening exercise and stretching in sub acute and chronic stage.
  • 55. SURGICAL MANAGEMENT  Surgical Treatment should be aimed at pain relief or increasing the patient's function. This include  Hip and knee joint replacements.  Arthrodesis or arthroplasty for joints such as the thumb PIP.  Release of joint contracture.  In arthritis mutilans, surgical intervention usually is directed toward salvage of the hand. Combinations of arthrodesis, arthroplasty, and bone grafts to lengthen the digits may be used.  The goal is to maintain the pinch mechanism of the thumb and the first 2 fingers.
  • 56. REACTIVE ARTHRITIS (REITER’S SYNDROME)  Aseptic inflammatory polyarthritis that usually follows nongonococcal urethritis or infectious dysentery  The classic triad nonspecific urethritis, arthritis conjunctivitis.
  • 57. EPIDEMIOLOGY  The incidence is about 30-40 cases per 100,000 adults  Enteric form affect males and females with the same frequency.  Venereally acquired form occurs in a male-to-female ratio of 9:1.  Most patients are aged 20-40 years
  • 58. PATHOPHYSIOLOGY  Usually triggered by a genitourinary or gastrointestinal infection.  Chlamydia trachomatis has been implicated.  The mechanism of the interaction of the inciting organism with the host (often HLA-B27 positive) leading to the development of reactive arthritis is not known.  Synovial fluid cultures are negative for enteric organisms or Chlamydia species.
  • 59.
  • 60.
  • 61. LABORATORY STUDIES  Elevated markedly acute phase reactants, including (ESR) and (CRP),during acute phase  Normocytic normochromic anemia  IgA antibodies to specific bacterial antigens  aseptic pyuria.  Synovial fluid analysis reveals a high white blood cell count, most often with elevated polymorphonuclear leukocytes acutely. Gram stain and culture results are negative
  • 62. LABORATORY STUDIES  Throat, stool, or urogenital tract cultures and serologic techniques for the detection of Chlamydia species, including PCR, could be considered.  HLA-B27 testing yields positive results in 65-96% of cases
  • 63. IMAGING STUDIES  Early in the disease, no abnormalities are found on radiograph.  In more advanced or long-term disease, periosteal reaction and proliferation at sites of tendon insertion can be observed.  Exuberant plantar spurs.  Sacroiliitis occurs in less than 10% of acute cases but may be observed in 50% of those patients with chronic severe disease.  Syndesmophytes are usually asymmetrical.
  • 64.
  • 65. TREATMENT  Nonsteroidal anti-inflammatory drug, such as indomethacin (Indocin).  Evidence of erosion or sacroiliitis, methotrexate and azathioprine have been shown to be helpful.  Doxycycline or an analog for up to three months in Chlamydia-induced reactive arthritis.
  • 66. TREATMENT  Disease-modifying antirheumatic drugs→ In patients with persistent symptoms, sulfasalazine in dosages of 1 to 3 g daily, have been useful  Intra-articular steroid injections in patients with large knee effusions can be helpful.
  • 67. ENTEROPATHIC ARTHRITIS  The association of arthritis with inflammatory gut disease has been termed as enteropathic arthritis. This type of arthritis is common in crohn’s disease, ulcerarative colitis, Whipple’s disease.  Incidence of enteropathic synovitis in Crohn’s disease is 20.7%, 11% in ulcerative colitis, and 90% of pt. with Whipple’s disease.
  • 68. CLINICAL FEATURES  Recurrent attack of acute synovitis monoarticular in most cases  Knee is the most commonly affected  Erythema nodosum  In Whipple’s disease Arthritis is rather variable  Pseudospondylitis characterized by absence of sacroiliitis and response to antibiotic therapy reported in association with Whipple’s disease.  Tendency of exacerbation of gut and joint disease together  Surgery abolishes arthritis in ulcerative colitis not in Crohn disease  About 16% patient shows radiological sacroiliitis, 6% have unequivocal sign of the disease.
  • 69. INVESTIGATION  No characteristic finding ,only excludes other condition.  In Whipple’s disease diagnosis is confirmed by PAS.  Positive macrophage in small intestinal biopsy specimen
  • 70. TREATMENT  NSAID in short course to control joint disease.  Aspiration of swollen joint and instillation of hydrocortisone produces rapid relief.  Gut disease is treated accordingly and should not be influenced by joint disease..  In ulcerative colitis Best therapy lies in control of gut disease.  Maintenance of spinal and thoracic mobility by regular exercise program.  Whipples disease usually response to prolonged antibiotic treatment usually with tetracycline. Arthritis abates as the gut disease is controlled. In persistence cases antibiotic regime should be revised.
  • 71. BEHCET’S SYNDROME  Is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement.  Internationally agreed diagnostic criteria are:- Recurrent oral ulceration plus any of the following two→  recurrent genital ulceration  eye lesion  skin lesion  pathergy test Arthritis of Behcet’s is not deforming and affect knees and ankle.
  • 72. CONCLUSION Despite active interest in spondyloarthritis in recent years there remains many unresolved issues. For instance, should the ultimate criteria –‘the index of truth’ continue to be radiological sacroiliitis or should it be presence of HLA B27. The answer to this could derived from a more global approach along the following line→ – Continued recognition of importance of characteristics clinical feature. – Continued recognition of importance of radiological sacroiliitis. – Continued recognition of importance of HLA B27. – More flexible approach to allow wide spectrums of disease to be included in this group.

Editor's Notes

  1. Gaenslens test  The test is considered positive if the patient experiences pain while this test is performed, Sacral thrust test:One hand is placed directly on the sacrum and is being reinforced by the other hand. Purpose is to apply an anterior shear force to both sacroiliac joints since the ilia are fixed by the examination bench. The test is positive if pain is reproduced in the sacroiliac region.
  2. While stabilizing the opposite side of the pelvis at the anterior superior iliac spine, an external rotation, abduction and posterior directed force is then slightly applied to the ipsilateral knee until the end range of motion (ROM) is achieved  Further a couple of small-amplitude oscillations can be applied to check for pain provocation at the end range of motion.
  3. The shiny corner sign is a spinal finding in ankylosing spondylitis, representing reactive sclerosis secondary to inflammatory erosions at the superior and inferior endplates (corners on lateral radiograph) of the vertebral bodies which are known as Romanus lesions
  4. The dagger sign is a single central radiodense line on frontal radiographs related to ossification of supraspinous and interspinous ligaments. Trolley-track sign on AP view = central line of ossification (supraspinous and interspinous ligaments) with two lateral lines of ossification (apophyseal joints); Bamboo spine on AP view = undulating contour due to syndesmophytes;
  5. Simmons osteotomy:Laterally through the fused posterior joints bone is removed, exposing the eighth nerve root, leaving beveled edges to oppose later. Now osteotomy is closed Posteriorly by fracturing the spine and spine is immobilized with plaster body jacket incorporating halo freeman. Neck is gradually extended with a halo. When some of cervical lordosis is achieved , an arthrodesis from T3 to occiput is carried out with the halo still in place.