Approach to joint pain in pediatrics,and treatment

1. APPROACH TO A CHILD WITH
ARTHRITIS
DR MANOJ CHANDRAKAR
M.D.
CHANDRAKAR CHILDREN CLINIC
BILASPUR

2. ARTHRITIS
Swelling or effusion or the presence of 2 or
more of following signs :
1. limitation of range of movement ,
2. tenderness or pain on motion
3. increased heat .
ARTHRALGIA
Joint pain without any signs of inflamation.
• Monoarthritis : only one joint involved
• Oligoarthritis : 1-4 joints during 1st 6 months
of disease
• Polyarthritis : >5 joints during 1st 6 months of
disease

3. ETIOLOGY
• Infection : arthritis (viral, septic , tubercular ),
osteomyelitis
• Trauma
• Acute rheumatic fever
• Rheumatological disorders – JIA, SLE,JDM,KD,HSP
• Scurvy
• Blood diseases – sickle cell anemia , hemophilia ,
leukemia
• Reactive arthritis
• Malignancies
• psychogenic

4. WHAT TO ASK ?
• Onset of symptom and duration of joint
involvement
• Age /Gender
• History of preceeding event
• Whether involvement of single or multiple
• Progression of symptoms/Recurrence
• Sick/ nonsick
• Trauma/ illness/drug intake
• Family history

5. Associated symptoms – Fever, rash, bleeding,
ocular symptoms ,blood transfusions ,any
deformity ,weight loss, glands
Relationship of pain to Activity And sleep
History of bleeding diathesis
Is child suffering from any blood vessels
diseased?
Dietary history

6. MODE OF ONSET AND DURATION OF JOINT
INVOLVEMENT
 Acute - : < 2 weeks(ARF, HSP,trauma,
transient )
 Subacute : 2-6 weeks
(reactive arthritis ,SLE, dermatomyositis , PAN,
leukemia, sickle cell disease , hemarthrosis )
 Chronic : >2 weeks
( JIA, tubercular ,psoriasis)

7. HISTORY OF PRECEDING EVENTS
 Sore throat / scarlet fever : Rheumatic fever
 GI symptoms : reactive arthritis , GI infections
like salmonella , IBD)
 Urethritis : Reiters disease
 Viral infections (rubella,mumps,chicken pox, IM,
hep B) : monoarticular synovitis
 Recent immunizations : Rubella
 Trauma : {septic arthritis, hemarthrosis,
effusions, strained ligaments , sprained
muscles, dislocation , or fractures}.

8.  RTI : transient synovitis ( self limiting
characterized by sudden onset pain in hips ,
thigh and knees )
 Pyoderma : septic arthritis
 h/o drugs ( penicillins, immunoglobulins,
antitoxins) : arthritis/ arthralgia

9. JOINT INVOLVEMENT
 Single joint : tubercular, septic,
monoarticular, JIA, trauma
 Multiple joints : systemic disease (
migratory in rheumatic fever )
 Small joints : JIA, sickle cell disease( hand-
foot syndrome ) , psoriatic arthritis ,
tubercular dactylitis
 Large joints : most of the conditions

10. H/O FEVER
 High grade fever with chills and joint swelling
: septic arthritis
 Long standing , low grade and continuous
fever : T.B.
 Prolonged high grade irregular fever with
remissions : idiopathic arthritis or SLE
 Prolonged fever with joint involvement :
leukemia

11. PAIN
 Location – localized to particular segment or
involve larger area
 Intensity – pain scale on 1 to 10
 Onset - ? Acute ,? Trauma, ? Insidious .
Acute pain with trauma usually associated
with fractures
 Pain worsen on activity – destructive joint
pain
 Pain with morning stiffness , improve with
activity – JIA

12.  Growing pain – b/l improves with massage and
disappear in the morning
 In arthritis , pain worsens on massage
 Pain in infection has no diurnal variation
 Flitting pain (migratory )- acute rheumatic
fever
 Gait and posture – disturbance with pain
 If sacroiliac or other axial joint – experience
inflammatory back pain and alternating buttock
pain

13.  Symptoms characteristics of inflammatory
back pain :
 1. pain at night with morning stiffness and
improving upon rising
 2. no improvement with rest
 3. improvement with exercise
 4. insidious onset
 5. good response to NSAIDS.

14.  H/O bleeding – scurvy ( bleeding from
gums)
 Bleed with hemarthrosis on trivial injury:
hemophilia
 Bleeding PR : HSP
 H/O blood disease – sickle cell anemia ,
leukemia, hemophilia
 Cardiac symptoms – exertional dyspnea ,
chest pain , palpitation : Rheumatic carditis

15. EXAMINATION
 Local examination of joints
 Examination of spine
 Examination of muscles
 Presence of rash /subcutaneous nodules
 Lymphadenopathy / sinuses
 Eyes
 Oral cavity
 Desquamation of fingers
 Focus of infection
 Organomegaly
 Cardiovascular system

16. INSPECTION
 How patient moves in room before and during
examination and various manuevres
 Balance, posture , gait pattern
 Skin rashes , café –au-lait spots , hairy patches
, dimples , cysts ,tuft of hair , or evidence of
spinal midline defects
 Body habitus – signs of cachexia , pallor , and
nutritional deficiencies
 Any spinal asymmetry , axial or appendicular
deformities , trunk decompensation and
evidence of muscle spasm or contractures

17.  Forward bending test – assess asymmetry
and no movement of the spine
 Any discrepancies in the length
 Muscle atrophy
 Range of motions of all joints , their stability
and any evidence of hyperlaxity

18. PALPATION
 Local temperature
 Tenderness
 Assessment for swelling or mass
 Spasticity
 Contracture
 Bone or joint deformity
 Evaluation of anatomic axis of limb
 Limb length

19. EXAMINATION OF JOINTS
 Joints examined for – swelling , pain ,
tenderness and range of movements
 arthritis/ arthralgia : single joint or multiple
joints
 Large joints in lower extremities (knee/ankle)
: oligoarticular JIA
 Small joints of upper & lower extremities :
Polyarticular JIA
 Spindle shaped fingers : rheumatoid
arthritis

20.  Diffuse swelling of entire dorsum of hand and
foot : sickle cell disease
 Scorbutic beading of costochondral junction :
scurvy
 In T.B hip joint : limb is flexed . Abducted and
medially rotated
 Pseudoparalysis (inability to move joint due to
severe pain ) : septic arthritis
 Examination of spine(kyphosis/scoliosis) : TB
spine

21.  Examination of muscles :
 1. wasting above or below the joint seen in
idiopathic arthritis or chronic joint
involvement ( disuse atrophy )
 Tenderness of muscles : dermatomyositis

22. SKIN RASH
 ARF : erythema marginatum
 sJIA : faint evanacsent macular rash
(salmon colored ) . Linear or circular ,
mostly over trunk and proximal extremities ,
non pruritic and migratory with lesions ,
lasting for < 1 hour . Koebners phenomenona
also present .
 SLE : butterfly shaped malar rash (spares
nasolabial fold)

23.  HSP : palpable purpuric rash over the
extensor aspect of extremities
 JDM : heliotropes rash over upper eyelids
 Leukemia : purpuric and ecchymotic patches
 Rheumatic fever : subcutaneous nodules
over extensor aspect of upper extremities
and suboccipital region

24. EYES
 Pallor and jaundice with symmetrical painful
swelling of hand and feet (hand- foot
syndrome) : sickle cell disease
 Iridocyclitis : JIA
 High fever with conjuctival injection :
Kawasaki disease

25.  Oral cavity : spongy gums ( scurvy)
swollen tongue and lips ( Kawasaki
disease)
 Desquamation of fingers : Kawasaki disease
 Organomegaly : hepatosplenomegaly ( collagen
disease, leukemias, disseminated TB)
 splenomegaly : sickle cell disease
 Focus of infection : (boils, abcess etc.. ) septic
arthrtitis .

26.  CVS : 1. hypertension (SLE)
 2. pericardial rub /pericarditis : RA,SLE
 3. myocarditis : RA and KD

27. SOME FINDINGS IN POLYARTICULAR JOINT PAIN
 Bone tenderness or chest pain : sickle cell
disease
 Coexisting tendonitis : gonoccoccal or
rheumatoid disease
 Conjuctivitis, abdominal pain , and diarrhoea
: reactive arthritis
 Fever and malaise : infection , gout ,
rheumatic , vasculitis

28.  Malaise and lymphadenopathy : acute HIV
infection
 Oral and genital ulcer : behcet disease
 Raised silver plaques : psoriatic arthritis
 Recent pharyngitis, and migrating pain : ARF
 Recent blood product / vaccination : serum
sickness
 Skin ulceration , rash , abdominal pain :
vasculitis
 Tick bites : lyme arthritis
 Urethritis : gonococcal or reactive arthritis

29. MONOARTHRITIS

30. ARTHRITIS IN CHILDHOOD IS
COMMON WITH MONOARTHRITIS
BEING MORE COMMON THAN
POLYARTHRITIS.
• Acute monoarthritis (< 2
weeks)
• Chronic monoarthritis (> 6
weeks)

31. ACUTE
MONOARTHRITIS

32. CAUSES
1. Septic arthritis
2. Reactive arthritis including the initial
presentation of acute rheumatic fever and
post infectious arthritis
3. Hemarthrosis
4. Traumatic joint effusion
5. Bone tumours and acute leukemia
6. Juvenile arthritis (systemic onset or
enthesitis related subcategories)

34. APPROACH
The key points to focus upon are :
1. child ‘well’ or ‘sick’?
2. history of trauma?
3. Features suggestive of infection, either
localised or systemic?
4. family history of bleeding diathesis?
5. nature of the onset?

35. CONSTITUTIONAL FEATURES
1. Fever,
2. sore throat
3. weight loss
4. loss of appetite
5. diarrhoea
6. urethral discharge,
7. history of sexual activity
8. history suggestive of uveitis
(painful red eye)
9. Rash

36. 7. WHAT IS THE NATURE OF PAIN AND/OR
STIFFNESS?
– Night pain  osteomas or malignancies.
– early morning stiffness improvement with
gentle mobility  inflammatory arthritis.
– Pain more in the evenings ,worsens after movement or
with exercise  mechanical pain.
– steroids
– retinoids
– anticonvulsants
 avascular
necrosis
 monoarthritis
 articular
manifestation of
lupus
– Chelation therapies 
arthropathy
8. Is there a history of
medications being taken

37. EXAMINATION
General Examination Pallor,rashes, palpable
purpura, peeling of the skin,
thickening of the skin,
conjunctivitis, icterus,
lymphadenopathy, nail
pitting,pigmentation, psoriasis,
oral ulcers, nodules
systemic examination Tachycardia / murmurs,
presence of chest infection,
or hepatosplenomegaly
Musculoskeletal
system
swelling, redness and soft tissue
involvement

38. INVESTIGATIONS
Laboratory investigations: Radiological evaluation
A.CBC,ESR, CRP
B.Coagulation studies
C.Blood culture
D.Viral titers
E.Anti streptolysin O titre:
ASO titre
F.Throat swab
G.Tuberculin test
H. Synovial fluid culture
X- ray-
wide joint Space
features of injury
features of osteochondritis ,avascular
necrosis , Deposits in joints,
Malignancy
Ultrasound - effusion and for diagnostic
aspiration
MRI - show features of synovitis ,effusion
,bone Edema ,osteomyelitis , tumor
Echocardiography: assist in diagnosis of
child with suspected Acute Rheumatic fever

39. CBC
 HB low ( SCD ,leukemia )
 Leukocytosis with polymorphonuclear
predominance ( septic arthritis )
 Leukopenia with lymphopenia ( SLE)
 Reticulocyte count increased ( SCD)
 Thrombocytopenia ( leukemia )
 Thrombocytosis( idiopathic arthritis )
 Plat. Count normal (HSP)
 Anemia of chronic diseases ( collagen vascular
disease, TB)

40.  ESR : increased (ARF, collagen disease)
decreased (sickle cell anemia )
Clotting factors : hemarthrosis with prolonged
clotting time
Mantoux test : TB
Xray – scurvy, TB, idiopathic arthritis
RA factor – polyarticular JIA
ANA seropositivity – increased risk of chronic
uveitis in JIA
Anti ds DNA – SLE

41.  ASO titre – recent streptococcal infection In
ARF
 CRP- raised in ARF
 Throat swab culture – ARF
 Urine examination – albumin aand hematuria (
collagen disease)
 USG and MRI – septic and TB arthrtitis ,
hemarthrosis
 Synovial fluid aspiration

42. THE COMMON CAUSES

43. PYOGENIC ARTHRITIS
1. True clinical emergency
2. Common infecting organism is staphylococcus
aureus, streptococcus species, pseudomonas
aeruginosa, pneumococci, neisseria
meningitidis gonococcus, escherichia coli,
klebsiella and enterobacter species
3. Onset of fever, malaise and signs such as erythema,
local heat and significant pain at affected joint are all
suggestive of a septic joint
4. Diagnostic is monoarthritis with regional
lymphadenopathy .
5. Delay in treatment can result in disastrous complications
6. Total recommended antibiotic course is for at least 6
weeks.

44. TRANSIENT SYNOVITIS OF THE
HIP
1. In young toddlers and up to 8 years
2. Well child with a history of mild upper respiratory infection
in the recent
3. Diagnosis of exclusion and a septic hip is an
important differential where the child is toxic, febrile
and has a significant pain
4. Settles with simple analgesia in 24–48 h, needs rest

45. REACTIVE ARTHRITIS
1. Infection with enteric organisms (shigella,
salmonella and campylobacter)
2. Very painful, usually relatively short-lived
3. Presence of HLA-B27 in the patient and a family
history of spondyloarthropathy appear to increase
the risk
4. Arthritis,urethritis and acute conjunctivitis are
well described to occur together. Triad
(Reiter’s syndrome)

46. POST STREPTOCOCCAL REACTIVE ARTHRITIS
(PSRA) AND
ACUTE RHEUMATIC FEVER(ARF).
1. Usually present with multiple joint involvement
2. Pattern of joint disease is fleeting in ARF and usually
not involved for more than a wk
3. diagnosis of ARF is established mainly on clinical
grounds
major criteria include (1) carditis, (2) polyarthritis, (3) chorea,(4) erythema
marginatum, and (5) subcutaneous nodules. The minor criteria include (1)
arthralgia (counted only when arthritis is not present), (2) fever, (3) elevated acute
phase reactants, and (4) an electrocardiogram showing prolonged PR interval
4. PSRA resolves over 6–8 weeks and has a less
dramatic response to NSAIDS as compared to
ARF
5. PSRA tends to occur sooner after a streptococcal
infection
than the arthritis of ARF (7–10 vs 10–28 days).
6. Articular outlook for both forms is excellent

47. ARTHRITIS AS PART OF SYSTEMIC
ILLNESS
Systemic infections Systemic vasculitic illnesses
1. Leptospirosis,
2. Brucellosis,
3. Mycoplasma,
4. Hepatitis B &C,
5. Enteroviral
6. Arboviral infections such
as chikungunya
1. Kawasaki disease and
henoch schnolein
purpura
2. Monoarticular
presentation is
described though less
typical
3. Children have large joint
involvement of the lower
limbs with angioedema
over the hands and feet.

48. MALIGNANCY
1. Diffuse hematological malignancy (leukemia,
lymphoma)
2. localised osseous malignancy (osteosarcoma/
Ewing's) can present as joint pain
3. Red flag signs are that the child is usually sick and
may have bone pain, night pain and/or back pain.
4. features that point to the diagnosis of a malignancy
are pallor, hepatosplenomegaly, lymphadenopathy
and bony tenderness
5. X-rays may show periosteal reactions and other
features of bony malignancy

49. HEMARTHROSIS
• Especially a male infant who has significant bruising
after trivial trauma, large hematomas after vaccination
or spontaneous large articular swellings that begin
abruptly and are very painful
• Recurrent hemarthrosis can damage the joint and
lead to chronic arthropathy needing synovectomy
and/or joint replacement in the long term.
• Prophylactic aggressive factor replacement therapy
• Instillation of corticosteroid, to prevent the
development of chronic changes in the effected joint

50. CHRONIC MONOARTHRITIS

51. Causes
1. Juvenile arthritis (systemic onset oligoarticular
,and the ERA subcategories.)
2. Chronic hemarthrosis
3. Malignancies/bone tumors
4. Infections such as tuberculosis
5. Miscellaneous disorders e.g., Sarcoidosis,
pigmented villonodular synovitis.

52. APPROACH
• Distinguish between the sick and well child
• Identify for presence of other pointers of chronic
disease.
• Some conditions can present as either as acute or
chronic monoarthritis
– hemarthrosis
– subcategories of JIA
– children with malignancies.

53. Sick child Well child
1. Partially treated septic arthritis
2. TB
3. Other infection-lyme
disease, brucellosis
4. Vasculitis
5. Sarcoidosis
6. Collagen vascular disease-eg SLE
7. Malignancy
8. Arthritis associated with other
chronic diseases- IBD, celiac
disease
1. OJIA( oligoarticular JIA)
2. Enthesitis related arthritis
3. Psoriatic arthritis
4. Pigmented villo-nodular synovitis
5. Mechanical injury/foreign body
e.g. Plant thorn synovitis

54. HISTORY
1. Morning stiffness
2. Night pains
3. Restriction of activities
4. Recent sore throat,
5. Gastroenteritis,
6. Red and painful eyes,
7. Chronic skin disease such as psoriasis
8. Significant trauma
9. History of tuberculosis (tb) or contact
10.Recent travel to an area endemic for lyme
disease, brucellosis or A history of tick bite

55. EXAMINATION
1. Skin examination
2. Formal ophthalmology evaluation by slit lamp for
uveitis
3. Musculoskeletal examination

56. INVESTIGATIONS
Laboratory investigations: Radiology
1. Mantoux test
2. chest X -ray for TB
3. ASLO titre
4. Lyme/Brucella serology
5. Bone marrow
aspirate for infection
6. Bone marrow
biopsy for
malignancy
7. ANA screen
1. Joint space narrowing
2. Brodie’s abscess is
characteristic of
osteoarticular TB
3. Bone Scan is
useful in picking up
partially treated
septic arthritis,
juxta- articular
osteomyelitis

57. THE COMMON CAUSES

58. OSTEOARTICULAR TB
• By direct invasion into a joint
• Or as a reactive arthritis termed poncet’s disease
(usually a polyarticular disease)where the tubercular
infection is at a distant site
• Significant pain as a result of muscle spasm
around the involved joint
• Contact history with TB is frequently obtained
• Definitive diagnosis is by synovial fluid culture of
mycobacterium TB (low yield) or PCR for
mycobacteriumtb (low sensitivity, high specificity)

59. OLIGOARTICULAR JIA
(OJIA)
• monoarticular presentation diagnosis of
exclusion
• joint should be involved for more than 6 weeks
• characteristic morning stiffness and mild pain
• Baseline tests are usually normal MRI will
show synovitis and effusion
• NSAIDS and Intra-articular steroids ,
aggressive approach with DMARDs
needed.
• articular prognosis is usually excellent

60. PIGMENTED VILLONODULAR
SYNOVITIS(PVNS)
• Rare cause of chronic monoarthritis
• Benign synovial hypertrophic condition
• Girls more than boys
• Painless recurrent large joint effusion with no systemic
signs and normal inflammatory markers
• MRI is diagnostic ,it shows hemosiderin deposits
• T/t intra-articular steroids, later surgical or
radioactive synovectomy.

61. POLYARTHRITIS

62. • Caused directly by an infectious
agent or indirectly by immune
mechanisms,
• May be a component of a systemic
disease process or may be idiopathic

63. Viral Parvovirus B19, Enteroviruses, Adenoviruses, Mumps, Rubella,
Varicella zoster virus, Hepatitis B, Coxsackie virus,
Cytomegalovirus, EBV, HIV.
Bacterial Staphylococcal and streptococcal infections, Neisseria
gonorrhae, Hemophilus influenzae; Bacterial endocarditis.
Other Infections Tuberculosis, Leptospirosis, Fungal infections, Brucellosis
Parainfectious
/ Reactive
HIV, Group A streptococcal infections, Salmonella, Shigella,
Yersinia, Campylobacter, Mycoplasma, Chlamydia
Rheumatological Juvenile idiopathic arthritis (JIA), Systemic lupus erythematosus
(SLE), Juvenile dermatomyositis (JDMS), Behcet syndrome
Systemic
Vasculitide
s
Henoch-Schonlein purpura (HSP), Kawasaki disease (KD),
Polyarteritis nodosa (PAN), Wegener’s granulomatosis
Spondyloarthrop
a thies
Juvenile ankylosing spondylitis (JAS), Psoriatic
arthritis Enteropathic arthritis
Miscellaneous Sarcoidosis, Drug/serum sickness reactions
CAUSES

64. HISTORY
• patient demographics, disease chronology,
inflammatory nature, progression, distribution of
joint involvement and extra-articular manifestations
• arthritis persisting for more than 6 weeks usually rules
out an infective pathology
• Age At the onset
Early childhood Mid-childhood Late childhood
1. Polyarticular JIA
(RF negative),
2. Kawasaki disease
3. Henoch
schonlein
purpura (HSP)
1. Juvenile psoriatic
arthritis
2. Juvenile
dermatomyositis
3. Polyarteritis nodosa
1. Juvenile ankylosing
spondylitis (JAS)
2. SLE
3. Polyarticular
JIA (RF
positive)
• Gout and crystal deposition disease are extremely
uncommon in childrens

65. • Sex
boys Girls
1. Vasculitides like KD and PAN
2. spondyloarthropathies like
inflammatory bowel disease and JAS
1. Many rheumatological disorders
(e.g. SLE, Polyarticular JIA)
•Onset of Disease
Acute Subacute or chronic
1. Septic arthritis
2. Arthritis associated with KD/HSP
1. Polyaticular JIA
2. Sarcoidosis
•Past History
reactive arthritis  recent diarrhea, acute conjunctivitis,
urethritis, and fever with or without
rash
systemic onset JIA  pyrexia of unknown origin and
history of having received multiple courses of
antimicrobials.

66. EXAMINATION
1. Common patterns of Articular Involvement
Small Joints Large joints Small and large joints
1. Viral arthritis
2. SLE
1. JAS
2. Reactive arthritis
1. Polyarticular JIA
2. Psoriatic
arthritis
(asymmetrical)
2. Topography and distribution
Disease Symmetry Axial involvement
Viral arthritis Symmetrical NO
Polyarticular JIA Symmetrical / asymmetrical NO
JAS asymmetrical YES
Psoriatic arthritis Usually asymmetrical YES/NO
SLE Symmetrical NO
Reactive arthritis asymmetrical YES/NO

67. 3. IS ANY PARTICULAR
JOINT INVOLVED
– acute dactylitis or distal interphalangeal
involvement 
psoriatic arthritis
– Enthesitis  juvenile spondyloarthropathies
4. Is there a joint deformity
– Deforming arthritis  polyarticular JIA
– non-deforming arthritis a/w SLE and IBD

68. PHYSICAL SIGNS TO BE LOOKED FOR
System Involved Physical Finding Diagnoses
1.Ophthalmologic 1. Uveitis
2. Conjunctival
injection without
exudate
1. JIA
2. KD
2.Dermatologic 1. Malar rash,
alopecia Oral
ulcers
2. Heliotrope rash,
Gottron papules
3. Polymorphous rash,
perineal
desquamation,
edema, and
erythema of hands
4. Evanescent
salmoncolored
rash
5. Palpable purpura
6. Nail pitting
onycholysis
1. SLE
2. JDM
3. KD
4. SJIA
5. HSP, SLE
6. JIA (psoriatic)

69. 3. Neurologic 1. Seizures, psychosis,
mood disorder, decline
in school performance
2. Stroke
3. Proximal muscle
Weakness
1. SLE
2. SLE, vasculitis
3. JDM, MCTD
4. Cardiovascular 1. New heart murmur
2. Pericarditis
3. Raynaud phenomenon
1. ARF, IE
2. SJIA, SLE, ARF
3. SLE, MCTD,scleroderma
5. Respiratory tract 1. Pleuritis
2. Acute or chronic
sinusitis, Pulmonary
nodules, or
hemorrhage
3. Interstitial lung disease
1. SJIA, SLE
2. GPA
3. SLE or scleroderma
6
.Gastrointestinal/
Genitourinary
tract
1. Weight loss or
poor growth
2. Diarrhea/hematochezia,
colicky abdominal pain
3. History of gastroenteritis
4. History of urethritis or
cervicitis
1. IBD, malignancy, SLE
2. IBD, HSP
3. Reactive arthritis
4. Reactive arthritis,
gonococcal
arthritis

70. INVESTIGATIONS
Investigation Abnormality detected Comments
Markers of
inflammation
↑ ESR,
↑ CRP,
↑ Globulins,
thrombocytosis
ESR and CRP elevation usually
indicate
activity (ESR may not always be
elevated)
Hemogram Normocytic normochromic
anemia, leucocytosis ,
thrombocytosis,
Eosinophilia,
haemolytic anemia(DCT+)
SLE may have leucopenia
and thrombocytopenia at
presentation
Urine routine Urinary sediment,
sterile pyuria,
hematuria proteinuria
Sterile pyuria seen in JIA and
KD(not be mistaken for UTI)

71. Synovial fluid
analysis/Biopsy
JIA can have a
markedly PMN
response (not be
mistaken for septic
arthritis)
1. Indicated if diagnostic problem;
2. Gram stain,
3. pyogenic and
mycobacterial cultures
Biopsy under
arthroscopy
Specific
investigations
1. RF,
2. anti CCP
antibodies
3. HLA B27,
4. ANA
5. ANCA
1. Polyarticular disease can be RF
+/−, ANA usually positive in
SLE,
2. HLA B27 for Juvenile
spondyloarthropathies
and reactive arthritis,
3. ANCA for systemic vasculitides
Other
investigations
1. KFT,
2. LFT,
3. X-ray,
4. ASO titres,
5. Throat swab,
6. HIV,
7. Viral serologies,
8. ECHO
1. Baseline metabolic profile in
all patients
2. other investigations are disease
specific

72. Common causes
of polyarthritis
TREATMENT
SPECIFIC THERAPY COMMENTS
Infectious/
Parainfectious
1. Viral infections - self
limiting;
2. antimicrobials for bacterial
infections;
3. NSAIDs in reactive arthritis
Patients with Rheumatic fever
require long term penicillin
prophylaxis
Rheumatological
disorders
1. NSAIDs
2. steroids and
immunosuppressive
therapy depending on the
specific disorder
Physiotherapy and occupational
therapy as important as drug
therapy
Systemic
Vasculitis
1. Immunoglobulin in KD
2. NSAIDs/steroids in HSP
and other vasculitis
Miscellaneous Supportive and definitive
treatment depending on
aetiology
1. Immunoglobulin therapy in KD can
prevent long term morbidity
2. prompt administration of steroids
in lupus can be life saving
Disease may evolve in time in any
category and patients need follow up

73. THE COMMON
CAUSES

74. RHEUMATIC DISEASES
• Systemic JIA (SJIA)
• Criteria for the Classification of Juvenile
Rheumatoid Arthritis
• Age at onset: <16 yr
• Duration of disease: ≥6 wk
• Onset type defined by type of articular involvement in the 1st
6 mo

75. International League of Associations for Rheumatology
Classification of Juvenile Idiopathic Arthritis (JIA)
• Systemic
– Arthritis in ≥1 joint with, or preceded by, fever of at least 2 wk
in duration that is documented to be daily (“quotidian”*) for
at least 3 days and
– accompanied by ≥1 of the following
» 1. Evanescent (nonfixed) erythematousrash
» 2. Generalized lymph node enlargement
» 3. Hepatomegaly or splenomegaly or both
» 4. Serositis
• Oligoarthritis
• Polyarthritis (RF-negative)
• Polyarthritis (RF- positive)

76. • Psoriatic arthritis
– Arthritis and psoriasis, or arthritis and at
least 2 of the following:
• 1. Dactylitis
• 2. Nail pitting and onycholysis
• 3. Psoriasis in a 1st-degree relative

77. • SLE
– in adolescence with low-grade fevers
– constitutional symptoms of anorexia, weight
loss, malar rashes, and painfulpolyarthritis
affecting both the large and small joints
– ANA titer is strongly positive
– nephritis, cytopenias, hypocomplementemia,
anti- dsDNA,and other autoantibodies
differentiates SLE

79. • systemic sclerosis and idiopathic
inflammatory myositis
– mild nonpainful arthritiS
Juvenile
dermatomyositis
Systemic sclerosis Mctd
1. Weakness
2. Heliotrope rashes,
3. Gottron papules
1. Sclerotic skin
changes
2. Raynaud
phenomenon
3. Skin and gum
telangiectasias,
4. Respiratory
symptoms,
5. GI tract dysmotility
1. Raynaud
phenomenon,
2. Myositis,
3. Polyarthritis
4. Sclerodactyly
5. Positive ANA titer
6. High titers of
anti-rnp
autoantibodies.

80. VASCULITIDES
KAWASAKI HSP
Prolonged fever
Bilateral non exudative conjunctivitis
Erythema of lips and oral mucosa
Changes in the extremities – edema,
peeling
Rash – non vesicular
Cervical lymphadenopathy –
unilateral
Palpable purpura
Age ≤20 yr at onset
Bowel Wall angina
granulocytes
on biopsy

81. VIRAL PATHOGENS
• Parvovirus B19 (most widely)
– causes fifth disease/erythema infectiosum
– self-limited exanthem
– diagnosis is made if circulating IgM
antibodies to parvovirus
– Treatment is supportive with NSAIDs

82. • Rubella vaccine
– Symptoms usually 2 weeks after vaccination
– Symmetric, migratory, and additive
arthritis typically resolve within 2 to 4
weeks
• Several herpesviruses /hepatitis B and
C/HIV

83. OTHER IMPORTANT DIAGNOSTIC
CONSIDERATIONS
• IBD
– 2 patterns of joint disease
– first pattern is involvement of the lower
extremity joints, especially the ankles and
knees
– peripheral arthritis tends to parallel the activity of
the GI tract inflammation
– second pattern is of axial involvement and is
often associated with HLA-B27 and little
relation to the GI disease
– Medical management is aimed at optimizing
control of the GI tract inflammation

84. • Malignancy
– Infiltration of the bone or synovium can
mimic polyarthritis
– ALL can cause polyarthritis as a result of
leukemic infiltration into the synovium
– Laboratory evaluation may show moderate to
severe anemia or an elevation of the ESR,
with a normal or low platelet count; a low
WBC count; or high lactate dehydrogenase or
uric acid levels.

85. THERAPEUTICS FOR CHILDHOOD
RHEUMATIC DISEASES
CLASSIFICATION THERAPEUTIC INDICATIONS
NSAIDs Etodolac
Ibuprofen
Naproxen
Celecoxib
Meloxicam
JIA
Spondyloarythropathy
Serositis
Cutaneous vasculitis
Uveitis
DMARDs Methotrexate
Leflunomide
HCQ
Sulfasalazine
JIA
Uveitis
SLE
APLA syndrome
TNF alfa antagonist Adalimumab
Etanarcept
Infliximab
JIA
Spondyloarthropathy
Psoriatic arthritis
Uveitis
Sarcoidosis
Modulate T cell
activation
Abatacept JIA

86. CLASSIFICATION THERAPEUTIC INDICATIONS
Anti CD 20 antibody rituximab SLE
Interlukin 1 antagonist Anakinra
canakinumab
Systemic JIA
Interlukin 6 antagonist tocilizumab Systemic JIA
Intravenous
immunoglobulins
IVIG Kawasaki
Juvenile
dermatomyositis SLE
glucocorticoids Prednisone
Methylprednisolone
Intraarticular
Prednisolone
ophtalmic
suspension
SLE
Juvenile dermatomyositis
Vasculitis
JIA
Sarcoidos
is uveitis
immunosuppresive Mycophenolate mofetil SLE
uveitis
cytotoxic cyclophosphamide SLE
Vasculitis
Juvenile dermatomyositis

87. PGALS
• A recently developed and validated tool is
the pGALS ( pediatric Gait, Arms, Legs,
Spine), which is a simple screening
examination that can be performed in a
few minutes(2minutes ) .

91. pGALS
Any pain Right knee
Any difficulty
dressing
NO
Any difficulty
in walking
Yes
Appearance Movement
Gait Normal
Arms Normal Normal
Legs Abnormal Abnormal
Spine Normal Normal

92. Case discussion

93. 9 year old ,female child, residing at
Raipur,hindu by religion and belonging to
upper lower socioeconomic status
Presented to V.S. General hospital with
complaints of
1.Difficulty in getting up from sitting
position 15 days
2. Drooping of left eye lid – 10 to 12
days
3. Difficulty in speaking - 8 to 10 days
.

94. No difficulty in breathing , no difficulty in
swallowing, no history of trauma , joint pain ,
altered sensorium , convulsion , no altered
bowel and bladder functions ,no history of
recent vaccination.
Past History : No history of similar complaints
No h/o major illness.
Family History :
No history of
major illness

95. Birth History : not significant
Immunization History : appropriate for age a/c to
national immunization schedule
Diet History : full family mixed diet ,
predominant veg. diet
Development History : appropriate for age till
now

96. EXAMINATION
Patient was conscious ,
Vitals :Temp.- normal ,
pulse rate- 86/min. ,
RR. -26.min,
BP. -98/66mmhg,
Spo2- 98%on air ,
Weight -21.9kg,
Height -122cm,
BMI -14.9,
SMR stage 1
Pallor present , ptosis in left eye
no clubbing, no edema , no lymphadenopathy

98. Locomotor examination
Tone – normal in all 4 limbs
Power – (1) upper limb
proximal joints - 3/5 in both
distal joints - 4/5 in both
(2) Lower Limb
Proximal joints - 3/5 in both
distal joints - 4/5 in both
Reflexes - superficial and deep reflex were present
Gover sign : +

99. SYSTEMIC
EXAMINATION
1. R.S.
2. C.V.S within normal limits
3. Abdomen
4. CNS - conscious , oriented to time
,place, person
speech – nasal twang present
memory preserved,
Right handedeness
cranial nerves - normal

100. Motor system : bulk of muscles – b/l equal
Tone – normal
Power – Upper Limb –
1. proximal – 3/5
2. distal - 4/5
Lower Limb –
1. proximal – 3/5
2. distal - 4/5
Reflexes were normal
No sign of meningial irritation
No involuntary movement ,
Gait - limping ,no other cerebeller sign

101. LOCOMOTOR SYSTEM
Neck – 1. flexion – 4/5
2. extension – 4/5
Shoulder – 1. flexion – 3/5 in both
2. extension – 3/5 in both
3. adduction – 3/5 in both
4. abduction - 3/5 in both
5. circumduction – complete but
not overhead abduction > 90
Elbow – 1. flexion – 4/5 in both
2. extension – 4/5 in both
Wrist - 1. flexion – 4/5 in both
2. extension – 4/5 in both
Pelvic /hip – 1. flexion – 3/5 in both
2. extension – 3/5 in both sss
3. adduction – 3/5 in both
4. abduction - 3/5 in both
circumduction – complete but range less
Knee - 1. flexion - 4/5 in both
2. extension – 4/5 in both
Ankle - 1. flexion – 4/5 in both
2. extension - 4/5 in both
Gover ′s sign - positive

103. Hemoglobin 11.2g/dl
Total count 16000/cm3
platelet 4.53lac
DC 46/39
Na 133 mmol/l
K 4.9 mmol/l
ESR 25 mm in 1st hour
CPK –total 3786 ↑↑u/l ( 26- 140 )
TSH 4.47uIU/ml
B12 505pg/ml
LDH 1680.9u/l↑ ( 200 – 450 )
SGOT 129.7↑ u/l ( 0 – 45 )
2Decho normal
INVESTIGATION

104. CSF picture Normal
ANA ++
NCV Normal
EMG s/o inflammatory muscle diseases

105. DIFFERENTIAL
DIAGNOSIS
1. AFP – polio and GBS
2. myositis
3. musculer dystrophy
4. myasthenia gravis
5. endocrinopathies
6. metabolic
7. trauma
8. muscle inflammation – SLE , JIA , mixed
connective tissue

106. Diagnosis :
Inflammatory muscle disease p/o juvenile
dermatomyositis
Positive findings:
1. symmetric proximal muscle weakness
2.Gottrons sign +, calcinosis
3. Ix – CPK (↑),LDH↑,↑ALT, EMG s/o inflammatory muscle
disease

107. Patient treat with prednisolone 2mg/kg/day with supplement
Follow up after 15 days , improve in gait ,improve in skin lesion
Methotrexate

108. JUVENILE DERMATOMYOSITIS
 M/C inflammatory myositis in children
 MyopathySymmetrical proximal muscle
weakness

 Vasculopathy  Skin Manifestations.

109. ETIOLOGY
 Multifactorial
 based on genetic predisposition and
 unknown environmental factor.
 HLA B8, HLA DRB1*0301, HLA DQA1*0501 and HLA DQA1*0301 are
a/w
 increases susceptibility to JDM.
 Possible pathogens : group A streptoccocus, upper respiratory infetion,
GI infecton ,coxsachie virus B , toxoplasma, parvovirus B19 etc.

110. EPIDEMIOLOGY
 Incidence approx. 3 cases /1million /yr
 Peak onset between 4 and 10 year and
there second peak in late 45-64 year.
 Male : female – 2:1
 Familial association present

111. CLINICAL FEATURES

112.  In our Patient , the sign and symptoms :

 1. Muscle weakness

 2. dysphonia

 3. skin lesions

 4. gottrons papule

 5. calcinosis .

113. GOTTRONS PAPULE
Bright pink or pale
,shiny, thickened or
atrophic plaques over
the PIP and DIP joints
and on knees,elbows
.

114. HELIOTROPE RASH
Blue-violet discolouration of
eyelids a/w periorbital
edema.

115. FACIAL ERYTHEMA
Facial erythema
crossing the
nasolabial folds
(in case of SLE , no
nasolabial
involvement)

116. SHAWL SIGN
 Erythema seen over chest and neck

117. MECHANIC HANDS

118. CALCINOSIS

119. DIAGNOSIS

120. MUSCLE BIOPSY

121. INVESTIGATION
 Muscle derived enzymes : cretine kinase, aldolase, aspartate
aminotransferase, ALT,
 LDH.
 ESR
 RA factor
 ANA
 EMG
 Muscle biopsy
 MRI

122. DIFFERENTIAL DIAGNOSIS
 Weakness without rash : polymyositis, infective myositis (influenza A,B
coxsackievirus B etc .), musculer dystrophies , myasthenia gravis, guillane
barre syndromes ,endocrinopathies
(hyperthyroidism,hypothyroidism,cushing syndromes, addisons diseases),
mitochondrial myopathies, metobolic disorders .
 Infections a/w muscle symptoms : trichinosis, bartonella, toxoplasmosis,
staphyloccocal pyomyositis.
 Blunt trauma , crush injuries

 Myositis : Vaccination ,drugs ,growth hormone and graftvs host disease.
 Rash of JDM confused with : eczema, psoriasis,malar rash inSLE ,other
rheumatic diseases.
 Muscle inflammation : SLE ,IBD,ANCA +vasculitidis, JIA ,mixed
connective diseases.


123. TREATMENT
 Corticosteroids ; mainstay of t/t . doses
2mg/kg/day
 Methotrexate
 IVIG
 Steroid sparing drugs
 Avoid sun exposure , sun protection cream
 vitD and ca++ supplementation

124. COMPLICATION
 Muscle atrophy cutaneous calcification
 lipodystrophy
 Aspiration pneumonia,
 respiratory failure
 GI bowel vasculitis ,
 ischemia ,
 perforation
 Calcinosis cause cellulitis and
 osteomylitis
 Steroids toxicity

125. PROGNOSIS
 Mortality rate dec. from 33% to 1% with use of
steroids
 Active symptoms dec .from 3.5 yr to < 1.5 yr
 At 7yr of follow up , 75% no residual activity,
25% have chronic weakness, 40 % have chronic
rash .
 Upto 1/3 need long term medication to control
diseases.

126. THANK
YOU