:)

1. DISEASE: WHAT IS IT? HISTORY SYMPTOMS / PX CRITERIA (IF THERE IS) LABS DIAGNOSIS (INITIAL /ACCURATE) TREATMENT EXTRA NOTES!!!

2. SERONEGATIVE
SPONDYLOARTHROPATHIES
(Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Enteropathic arthropathy)
Ankylosing spondylitis:
- Inflammatory disorder of the axial skeleton and
the peripheral joints (Shoulder, hip)
- Distinct from most autoimmune disease in that it
is predominant in young men who are “under
the age of 40”
- MEN affected more than women by about 4:1
Reactive Arthritis:
- Inflammatory arthritis occurring after or during
a bacterial infection
- Two types which presents similarly:
- Post Diarrheal:
- occurs after infection with:
- Campylobacter
- Salmonella
- Shigella
- Reiter’s syndrome:
- occurs during or after: -
- nongonococcal urethritis
Ankylosing spondylitis:
N/A
Reactive Arthritis
History will be very important: for instance,
arthritis (>1 month) + urethritis has almost 99
% specificity for Reiter’s syndrome
Psoriatic Arthritis (PsA):
Ankylosing spondylitis:
- The primary complaint tends to
be persistent and chronic
refractory lower back pain
* worst in the morning and improves
throughout the day (like most
autoimmune arthropathies)*
PX:
- On examination patients will
have a loss of lumbar lordosis
which indicates the
characteristic fusion of lumbar
spine processes (this may make
the patient susceptible to spine
fracture)
Reactive Arthritis:
- Primarily affects:
- weight bearing joints
(knees, hips, lower back)
- Uveitis
Ankylosing spondylitis:
N/A
Reactive Arthritis:
N/A
Psoriatic Arthritis:
Classification criteria for psoriatic arthritis
(CASPAR):
If the patient has 3 or more points there is > 90
% sensitivity and almost 99 % specificity for
psoriatic arthritis
1. Family history of psoriasis:
a. Current (2 points)
b. Previous (1 point)
2. Psoriatic nail changes (1 point)
Ankylosing spondylitis:
N/A
Reactive Arthritis:
N/A
Psoriatic Arthritis (PsA):
N/A
Enteropathic Arthritis:
N/A
Ankylosing spondylitis:
The best first step is: based on clinical
symptom and on lumbar spinal XR
Schober’s test:
- A useful clinical tool for detecting a
loss of lumbar lordosis and therefore
possibly ankylosing spondylitis
- An increase of < 5 cm is considered a
positive Schober’s test and points to a
pathologic process
Reactive Arthritis:
- Can be made clinically.
- Should attempt to find the source of
the infection
- Patients with a history of diarrhea
within the last 2-6 weeks can be
considered to have:
Ankylosing spondylitis:
The best first step in therapy of patients with AS is:
- NSAIDs (Indomethacin; Ibuprofen)
- Physical therapy.
SECOND LINE AGENT:
- TNF inhibitors (infliximab, adalimumab, etanercept)
SURGERY:
- Hip replacement surgery
- Lumbar Spinal Osteotomy
Reactive Arthritis:
- NSAIDS are the cornerstone of therapy
- *Reactive arthritis and Reiter’s syndrome are self- limited*
Reiter’s Syndrome:
- a 3 months course of tetracycline may be of benefit
Derm symptoms:
- Topical corticosteroids (betamethasone)
Uveitis:
- Optho consultation
All seronegative spondyloarthropathies
share one thing in common: they all share
the presence of HLA B-27 antigen
(from the mutation on chromosome 6)

3. (Chlamydia trachomatis)
Psoriatic Arthritis (PsA):
- Inflammatory, erosive arthritis associated with
the skin condition psoriasis
Risk Factors:
- Personal and Family history of psoriasis.
- White > blacks, Hispanics
- MOA is around 35-50 years
Enteropathic Arthritis:
- Arthritis that is accompanied by and particularly
associated with flares of IBD
- About one in five people with Crohn's or
ulcerative colitis will develop enteropathic
arthritis
N/A
Enteropathic Arthritis:
N/A
- Dactylitis
- Low back pain
- Circinate balanitis
- Small, hard nodules on soles of
feet
- Possible continued symptom of
infection (diarrhea, dysuria)
PX:
1. Knee joint may be:
a. Warm
b. Red
c. fluctuant
2. In that case the best initial step
is to get a:
a. synovial analysis
3. Synovial fluid will be high for
WBCs,negative culture
Psoriatic Arthritis (PsA):
3. Dactylitis (1 point)
4. Radiographic changes (1 point) :
a. Osteophytes
b. joint erosion
5. Negative RF (1 point)
POOR PROGNOSIS:
1. Extensive skin involvement
2. A strong family history of psoriasis
3. Female gender
4. Disease onset at < 20 years of age
5. Expression of HLA-B27, -DR3 or -DR4
alleles
6. Polyarticular or erosive disease
Enteropathic Arthritis:
- a post diarrheal etiology
- Patient without a history of diarrhea
with a questionable sexual history
should consider a :
- throat and urogenital culture/
- PCR for Chlamydia
Psoriatic Arthritis:
Criteria based
Enteropathic Arthritis:
N/A
- Generally the treatment is ophthalmic corticosteroids (prednisolone)
Psoriatic Arthritis:
1. NSAIDs are the first line of therapy
2. Methotrexate is second line
3. Etanercept is approved for monotherapy
Enteropathic Arthritis:
1. NSAIDs
2. 5 – ASA derivatives (Sulfasalazine,pentasa)
3. Biologic medications, the TNF inhibitors

4. 1. Plaque psoriasis (silver scaling,
erythematous plaques, guttate
lesions, pustules)
2. Psoriatic nail changes
3. Dactylitis (inflammation of the
digits, especially the DIPs)
Enteropathic Arthritis:
Axial or peripheral joint pain in a
patient with symptom of IBD
(Chronic diarrhea, cramping, mucus in
stool, tenesmus, oral aphthous ulcers)
Arthritis symptoms may precede the
IBD symptoms
N/A

5. OSTEOARTHRITIS Chronic, progressive, non- inflammatory ,
idiopathic, degenerative, joint disease
Primarily OA: Closely related to the aging process
Other arthropathies can predispose to OA
Cause of secondary OA:
- WEIGHT!!!!!
MOST COMMONLY AFFECTS:
- joints of the:
- Hands
- weight - bearing joints (knees, hips)
SYSTEMIC→
- Primary OA is NOT associated with any systemic
symptom
LOCATION→
- Hands (wrists, MCPs, PIPs, DIPs)
- Weight bearing joints (hips, knees)
INFLAMMATION→
- *JOINTS ARE NOT EXTERNALLY INFLAMED
(NO SIGNS OF INFLAMMATION)!!!*
Joint pain is most common presenting
complaint.
Patient tend to be older (50+)
Secondary OA Risk Factor is:
- Obesity
- Joint trauma
- Repetitive joint work
- and other arthropathies are risk factors
Primary Risk Factor:
- Usually Idiopathic
Patient’s will often complain of pain in
knees, hips, and hands
Absence of constitutional symptom
(no fever, out of the ordinary fatigue,
malaise,etc)
*REMEMBER: OA IS NOT AN
INFLAMMATORY CONDITION!!*
PX:
- Joint often appear normal,
externally.
- Decreased range of motion
(ROM)
- Crepitus→ osteophytes
- Nodes on DIPs and PIPs (both
nodes are present)
ALL LABS ARE NORMAL
*Abnormalities in labs are not
indicative of osteoarthritis*
The first step in diagnosis is X Ray of the
affected joints
Typical findings is a reduction in joint space
Therapy is palliative:
- Reduce pain
- Maximize functional capacity.
• NSAIDs are the mainstay of treatment !!! (ibuprofen, ketoprofen, meloxicam, diclofenac)
• Acetaminophen is the treatment of choice !!!
Occupational therapy
Weight reduction
Hot/cold compresses
Exercise
Physical therapy

6. CHRONICITY→
- chronic and progressive.
- Insidious onset.
- Worsens with activity, pain improves with rest
EVIDENCE OF TRAUMA→
- May occur secondary to traumas to specific
joints.
- Primary OA is not associated with trauma

7. RHEUMATOID ARTHRITIS Chronic inflammatory, systemic disease highlighted
by severe joint pain.
Women > Men
Progressive
Can dramatically reduce quality of life
Location :
- particularly in hands, but NOT in DIPS (distal
interphalangeal joint)
Systemic?
YESS!!!!! Most patients will have a history of:
- Fatigue
- Malaise
- Weakness prior to the onset of joint pain
- Fever
- Fatigue
- Malaise
Multiple systemic complications:
- Anemia of chronic disease (ACD)
- Felty’s syndrome (due to splenomegaly,
no pancytopenia)
- Baker’s cysts
Felty's syndrome:
- is a complication of long-standing
rheumatoid arthritis
- defined by the presence of three
conditions:
- Rheumatoid arthritis
- Splenomegaly
Technical diagnostic criteria
(ACR) Must fulfill at least 4 for diagnosis:
1. Morning stiffness of affected joints for > 1
hr X 6 weeks
2. Swelling of hand joints x 6 weeks
3. Symmetric joint swelling X 6 weeks
4. Subcutaneous nodules (Rheumatoid
nodules)
5. Positive rheumatoid factor (RF)
6. Deformities noted on radiography
Based on:
- Clinical
- Imaging
- Laboratory criteria
There is no one pathognomonic test
- positive RF
- Anti -cyclic citrullinated peptide
(anti-CCP) ( more sensitive but more
expensive)
*antibodies are highly suggestive*
The best initial diagnostic step is
radiography (XR is fine, CT is more
accurate)
*Xray first, CT more expensive*
Rheumatoid factor should be drawn as well
*Note: if patient has only one inflamed joint
or the inflamed joints are asymptomatic, a
synovial fluid test may be the best first step*
Metotrexate (MTX) *It can cause hepatic fibrosis*, with or without a biologic
agent
*NSAID, and steroids are not disease modifying*
Biologic agents:
- Primarily TNF- a inhibitors.
- Greatly reduce general inflammation
- Infliximab
- Etancercept
- Adalimumab
- Adverse effect:
- Immunosupression
- Before starting a patient on a biologics get a Tuberculosis test (PPD test) to
rule out latent TB
Newly diagnosed patients may be treated with an initial short- term course of:
- corticosteroids (prednisone), to bridge over, as MTX and biologics take effect
NSAIDs can be used for pain relief, but not alter disease course
Rheumatoid arthritis is associated with a high risk for morbidity and
premature death secondary to the earlier development of cardiovascular,
lung diseases and malignancy
Boutonniere deformity is a deformed position of the fingers or toes, in which
the joint nearest the knuckle (the proximal interphalangeal joint, or PIP) is
permanently bent toward the palm while the farthest joint (the distal
interphalangeal joint, or DIP) is bent back away (PIP flexion with DIP
hyperextension).
COMPLICATIONS:
- Adverse effects from medications
- Progressive reduction of quality of life
- Felty’s syndrome
- Baker’s cysts
- Carpal tunnel syndrome :
- Casting
- Immobilization
- Atlanto-axial subluxation :
- patient should be advised to caution anything that involves
intense cervical motion
- Symptom of impingement vary from hand/ foot paresthesias to
quadriplegia

8. - An abnormally low white blood cell
count.
Baker’s cyst:
- is a fluid-filled growth behind the knee.
- It causes a bulge and a feeling of
tightness.
- Also called a popliteal cyst.
- A Baker cyst sometimes causes pain.
- The pain can get worse with activity or
when fully
- Straightening or bending the knee
- Knee damage caused by a sports injury or
a blow to the knee can lead to a Baker's
cyst developing.
- Rheumatoid arthritis
- Osteoarthritis
- Gout
If a synovial fluid analysis is ran on a patient
With Rheumatoid Artheritis there will be:
- elevated WBCs not elevated enough
to suspect septic arthritis
- If suspected, first step is always to stabilize patient and get a
plain radiograph of the neck
*ACUTE PHASE REACTANTS: AFTER FELTY SYNDROME*
Although RA is more common in females, extraarticular manifestations of the
disease are more common in males !!!
The extra-articular manifestations of RA can occur at any age after onset
characterized by destructive polyarthritis and extra-articular organ
involvement, including the:
- Skin
- Eye
- Heart
- Lung
- Renal
- Nervous and gastrointestinal systems

9. Baker's cysts usually develop in people aged 30
to 70, although they can affect people of any
age, including children.

10. POLYMYALGIA RHEUMATICA ● ALWAYS SEEN IN >55
● TEMPORAL ARTERITIS CORRELATION
● IDIOPATHIC
● INFLAMMATORY DISORDER OF PAIN
● ASSOCIATED WITH PROXIMAL MUSCLES.
*PAIN*
● ABRUPT ONSET
● SELF LIMITED
*the key feature is pain, and not too much weakness*
Whites are affected more than blacks 2:1.
Woman affected more than men
There is a high correlation between polymyalgia
rheumatica and temporal (giant cell) arteritis.
This is a syndrome of the elderly (50+)
Nearly half of patients with temporal (giant cell)
arteritis have, or will have polymyalgia
rheumatica!!
Patinent will describe:
- General aches and pain
- Stiffness
- Especially of the upper arms
- Shoulders
- Hip girdle
The pain tends to be worst in the
morning
PX:
- Normal strength (however
exerting more strength may elicit
pain)
- Active range of motion is limited
by pain
Clinical and of exclusion
- The best initial test would be an ESR ,
which would be elevated * START THE
TREATMENT RIGHT AWAY PLEASE*
Oral corticosteroids, namely, prednisone
Patinet typically have a rapid response to corticosteroid (Then asses if patinet has teporal
arteritis via biopsy)
The steroids can be tapered down to the lowest effective dose
All patients should be assessed for symptoms related to temporal
arteritis, due to the high association
Patienst with symptomsof temporal arteritis will require:
- a temporal artery biopsy for confirmation and consultation with an
ophthalmologist.
However, the treatment (w/prednisone) is the same
Imagining does not play a significant role in diagnosing PR.
However, MRI has shown that the inflammation is more
around the bursa and tendon, rather, than inside the muscle
Itself

11. FIBROMYALGIA Chronic , non- inflammatory , “pain processing”
disorder
Syndrome of:
- Widespread pain
- Stiffness
- Fatigue
- Disrupted sleep
Often accompanied by:
- Mood or anxiety disorders
- Disturbances in memory and other somatic
disorders (Irritable bowel syndrome, overactive
bladder)
Increased incidence in patients suffering from
“autoimmune disorders”
Increased in patients who have suffered:
- Psychiatric or physical trauma (may start
disease)
Majority of the patients between the ages of 35 and 60
years
Symtom/ PX: Patient will appear tired
*Pain is illicitable from characteristic
“pressure points” which are mandatory
for diagnosis !!!*
A basic lab profile:
- CMP
- CBC
- TFT
- ESR
- A mental health exam
will be of use
ENTIRELY CLINICAL !!!
1. It is prudent to rule out or vigilant for
any other alternative/co- existing
disorders
2.
Symtomatic Treatment
All patients diagnosed with fibromyalgia should be prescribed a medication for the pain
The most common drug used is a TCA *for pain killers* (amitriptyline)
Recently, gabapentin and pregabalin have been used *More effective but more expensive*
Sleep medications:
- Zolpidem (reduces fibromyalgia fatigue, but not fibromyalgia pain)
*Patient requires psychiatric evaluation/ consultation)
Patients often complaints of “ hurting all over” “feeling like I always have the
flue”, persistent fatigue, co- existence of other somatic problems such as IBS
or overactive bladder
TRIGGER POINTS:
- 18 points (9 pairs) tend to be painful when pressed, and may spread
pain to other body parts.
People with fibromyalgia have pain in at least 11 of these tender points when
a doctor applies a certain amount of pressure

12. Women are more likely to be diagnosed with
Fibromyalgia
May be difficult to diagnose and is often a diagnosis of
exclusion !!!

13. SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE)
- Chronic, inflammatory, multisystemic disease in
which arthritis is one of a myriad of possible
symptoms and presentations
- Idiopathic
- UNKNOWN ETIOLOGY AND SOME GENETIC
PREDISPOSITION
- Blacks and Hispanics are affected at higher rates
than whites
- Women are affected more than man by a 9:1
ration
- Age of onset:
- < 16 years (20%)
- >16 to 55 years (65%)
- > 55 years (15%)
Major causes of mortality:
- Infections and nephritis (early)
- Atherosclerosis (late)
Genetics:
- HLA-A1
- HLA-B8
Younger female, often of color presenting with
facial rash and/or joint pain
Be sure to get OB history
Anti - RO antibodies
- Fever
- Chills
- Headache
- Fatigue
- Malaise
- Weight loss
Renal:
- Hematuria
- Protenuria
Skin:
- Malar “Butterfly” rash
- Photosensitivity rash
Cardiac:
- Myocarditis
- Pericarditis
- Endocarditis
- Atherosclerosis
- Pancarditis
CNS:
- Conginitive dysfunction
DIAGNOSTIC CRITERIA FOR SLE
(SOAP BRAIN MD)
Four diagnostic criteria satisfies
diagnosis
S- Serositis
O- Oral Ulcers
A- Arthritis
P- Photosensitivity *avoid sunlight =,
because it can aggravate and cause
B- Blood D/O
R- Renal Abnormalities
A- ANA+ ⇒ not Gold Standard
I- Immunological phenomenon (anti-
Sm, AntidsDNA) *GOLD STANDARD*
Ultimately a diagnosis based on criteria and
supported by laboratory evidence
the best initial diagnostic test is ANA
*ANA IS NOT THE MOST ACCURATE*
Patients who are ANA + should get
Anti-dsDNA and Anti- Sm serologies, as
they are more specific (HALLMARK)
Focused on controlling symotoms
Arthralgia/pleuritis/ inflammation:
- NSAIDs (Ibuprofen, ketorolac, etc)
Rash:
- Corticosteroid topical ointment (betamethasone)
*Preventative care and corticosteroids (prednisone) should be used when
symptoms become frequent and certainly if patient develops renal
insufficiency*
In severe cases (severe lupus nephritis, heart/lung/ CNS involvement)
patients should be started:
- Cytotoxins (azathioprine, cyclophosphamide)

14. - HLA-DR
*Classic triad:
- Fever
- Arthralgia
- rash (malar, discoid, photosensitive)
Pregnancy and SLE:
- At high risk for spontaneous abortion
- This is a manifestation of anti- phospholipid
antibody syndrome
- All pregnant woman should be screened during
pregnancy for Anti- Ro antibody
- Babies born to Ro+ mothers are at risk for
neonatal lupus , which typically manifests with a
complete heart block
- All drug should be stopped during first
trimester, After first trimester patient should be
started on lowmolecular weight heparin and
aspirin
NEONATAL LUPUS
Pulmonary:
- Pleurisy
Musculoskeletal:
- Myalgias
- Arthralgias
- Arthritis
Hematologic:
- Anemia
- Leucopenia
- Thrompicytopenia
- Pancytopenia
Lymphatic system:
- Lymphadenopathy
OB/GYN:
- History (Spontaneous Abortions)
N- Neurologic symptoms not explained
otherwise
M- Malar Rash *Butterfly*
D- Discoid Rash
In emergent flares (RP GMN, severe neuro symptom):
- iv corticosteroids and cytotoxics should be used
Belimumab is the one biologic that is approved for use in SLE

15. Clinical Manifestation:
- Cardiac:
- Conduction defects
- Complete heart block rhythm
abnormalities
- Dermatologic:
- Erythematous papules
- Annular plaques
- Hematologic:
- Thrombocytopenia
- Leukopenia
- Anemia
- Hepatic:
- Hepatitis
TREATMENT:
- Involves:
- Surgical implantation of a pacing device
- Symptomatic control
- Generally with hydrochloroquine and IV

16. or topical steroids
NSAIDS SHOULD BE AVOIDED => MAY DEVELOP
RAYE’s SYNDROME
RESOLVES BY 6-8 MONTHS OF AGE!!

17. MYOSITIS
( POLYMYOSITIS &
DERMATOMYOSITIS)
Idiopathic (slow- onset, progressive), autoimmune,
inflammatory disease of skeletal (and
occasionally cardiac) muscle
Idiopathic myositis (Polymyositis, Dermatomyositis,
Inclusion body myositis):
- Age of onset is around 45-60 years
- Increased incidence with other autoimmune
diseases
- Best first diagnostic step is:
- Creatine phosphokinase (CPK) and
Aldolase
- Most accurate test is:
- biopsy and it is absolutely necessary to
establish the diagnosis
POLYMIOSITIS & DERMATOSIOTIS:
Women are affected more than men 2:1,
Blacks more than whites 5-8:1

18. *For inclusion body myositis men affected more
than woman*
POLYMYOSITIS:
1. Idiopathic, autoimmune and inflammatory
2. Affects proximal muscles most dramatically
3. Symmetrical
4. Distal muscles are relatively conserved
5. Ocular muscles are NEVER involved
6. Prognosis is good: main complications are:
a. dysphagia
b. interstitial lung disease
*A high creatinine kinase result would suggest
Polymyositis, CK is released when muscles breakdown
which occurs in polymyositis*
*Symmetrical muscle weakness affecting big muscles
is seen in polymyositis*
Polymyositis:
- Progressive Weakness
- Problems with ADL (activities of
daily living) including:
- brushing hair
- getting out of a chair
- holding head up
- carrying objects
- lifting arms up over head
Symptoms will be worst in the
morning, and improve throughout the
day
May present:
Weight loss
Fever
Hyperhydrosis
Generalized hyperhidrosis
Chronic pain
POLYMYOSITIS:
1. best first step in the
management/diagnosis:
a. CPK and aldolase levels
*These are markers for
muscular inflammation*
2. The most accurate test is:
a. muscle biopsy *Do this if
CPK/Aldolase are high*
3. It is judicious to order titers for Anti-
Jo1
a. Patient + for this antibody are
at increased risk to develop
interstitial lung disease
POLYMYOSITIS:
Best initial treatment for polymyositis is:
- Corticosteroids, (prednisone), If prednisone alone is not effective, cytotoxics or
biologics may be added
ADULT POLYMYOSITIS: Rituximab
DERMATOMYOSITIS:
Rituximab in the Treatment of Refractory Adult and Juvenile Dermatomyositis

19. DERMATOMYOSITIS:
Idiopathic, autoimmune and inflammatory disease
Also affects the skin.
1. Affects proximal muscles most dramatically
2. Symmetrical
3. Distal muscles are relatively conserved.
4. Ocular muscles are NEVER involved
INCLUSION BODY MYOSITIS:
Hereditary or acquired inflammatory and pathologic
disorder of muscle.
Inclusion body myositis may be asymmetrical
Affect distal muscles (hands, fingers) more notably
Fatigue
Dyspnea
Malaise
DERMATOMYOSITIS:
*same as polymyositsi*
- Progressive Weakness
- Problems with ADL (activities of
daily living) including:
- brushing hair
- getting out of a chair
- holding head up
- carrying objects
- lifting arms up over head
May present:
Weight loss
Fever
Hyperhydrosis
Generalized hyperhidrosis
Chronic pain
Fatigue
Dyspnea
Malaise
1. The best first step is:
a. CPK and aldolase levels.
2. The most accurate test is:
a. muscle biopsy (do this if
CPK/Aldolase are high)
Inclusion Body Myositis:
- Is refractory to steroids and immunosuppressants.
- Not deadly
-
- Early stages of disease can be confused w/ amyotrophic lateral sclerosis.
The muscle biopsy will confirm Inclusion body myositis
Unlike Inclusion body myositis amyotrophic lateral sclerosis is fatal

20. Affects men more than woman In addition, dermatomyositis will
present with the characteristic
heliotropic rush (face, eyelids,
sunexposed areas)
2 SIGNS & 1 RASH :
GOTTRON’s SIGN:
- purple/pink papule over the
Metacarpophalangeal (MCP) and
proximal interphalangeal (PIP)
joints.
HELIOTROPE RASH:
- purple/pink rash around the
eyes - eyelids may be swollen.
SHAWL SIGN:
- rash over the upper back,
shoulders and a V on the chest
INCLUSION BODY MYOSITIS:
INCLUSION BOSY MYOSITIS
1. The best initial diagnostic step:

21. - Insidious asymmetrical
weakness:
- Dysphagia
- Distal and proximal
weakness,
- Droopy head
- Facial muscle weakness
- No ocular involvement
Px: Decreased tendon reflexes are
common
a. CPK and aldolase
2. The most accurate test is:
a. Biopsy
*Electromyography (EMG) may be ordered*

22. WEGENER’S GRANULOMATOSIS Auto-immune inflammatory systemic disease of the
SMALL VESSELS!!
Commonly implicated by C- ANCA
Involves: Organ System, but most prolly:
Respiratory tract
Skin
Kidneys
History of chronic sinusitis that is refractory to
therapy
Refractory chronic sinusitis
“Saddle nose” deformity
Hemoptysis
Dyspnea
Polyarticular arthritis
Palpable purpuric rash
N/A Elevated ESR
Elevated CRP
Normocytic Anemia
Abnormal UA
The best initial test: an ANCA (c-ANCA) titer
The most accurate test is: Renal Biopsy
*shows granulomas*
Prednisone and cyclophosphamide are the mainstays of therapy
After 18 months, Cyclophosphamide
( which cannot be used more than 18 months) can be replaced with Methotrexate or
Azathioprine
Among the vasculitis in that the upper respiratory symptoms tend to take
central stage
Note: the lungs will always be involved even if the patient is asymptomatic.
CXR or CT (more accurate but may not be necessary) will show nodules
*ONLY CYCLOPHOSPHAMIDE REPLACES*
BONUS: What is an adverse effect of Cyclophosphamide?
Patient’s who are on cyclophosphamide therapy should get prophylaxis for
Pneumocystis carinii pneumonia
• TMP/SMX or (dapsone or atovaquone)

23. MICROSCOPIC
POLYANGIITIS
Auto- immune inflammatory systemic disease of the
SMALL VESSELS!!
Commonly implicated by P-ANCA
Can involve any organ system, but most
prolly involved are:
Skin
Lungs
Kidneys
Neuromuscular system
*MULTISYSTEMIC*
Most patient’s will describe a history of
frequent:
Muscle aches and pains
Fatigue
Weakness
Malaise
Weight loss
Hemoptysis
Mononeuritic multiplex:
(Inflammation of two or more
nerves, typically in unrelated parts of
the body)
Palpable purpuric rash:
N/A
Elevated ESR
Elevated CRP
Normocytic Anemia
Abnormal UA
The best initial test: an ANCA (p- ANCA)
titer
The most accurate test is biopsy
NOT IN PPT????? Note: Microscopic polyangiitis can be conclusively
differentiated from Wegener’s granulomatosis on
biopsy
No granulomas SEEN
POLYARTERITIS NODOSA Autoimmune inflammatory systemic disease of the
MEDIUM VESSELS, particularly those in the muscle
Can involve any organ system, but uniquely PAN spares
the lungs (NEVER INVOLVED)
Most commonly involved:
Skin
Kidney
Nerves
N/A Neuro/neuromuscular symptoms are
most prominently present in 70% of
patient which includes:
Peripheral neuropathy
(pins and needles)
Transient loss of vision
Patient must have 3 of the following 10 criteria :
1. Weight Loss of 4 KG or more
2. Livedo reticularis (Mottled skin)
3. Testicular pain or tenderness
4. Myalgia or leg weakness/tenderness
Elevated ESR
Elevated CRP
Normocytic Anemia
Abnormal UA
Best initial diagnosis & most accurate test is
a biopsy
*This can be done at a skin ulcer, at the
sural nerve or skeletal muscle.*
*If a biopsy cannot be undertaken,
angiogram is ok (i.e renal angiogram).
Prednisone and Cyclophosphamide *Postprandial GI pain is not uncommon*
*Hypertension secondary to renal factors is common too (restricted arterial
flow to kidneys activates RAAC*
*In patients with HBV & HCV, cyclophosphamide CAN NOT BE USED!!!*
Cyclophosphamide may be substituted with Methotrexate or Azathioprine in
18 months (remission

24. GI tract (Postprandial abdominal pain)
Associated with:
Hep B
Hep C
Mononeuritis multiplex
(Inflammation of two or more
nerves, typically in unrelated parts of
the body)
The skin rash is similar to WG and MPA,
however it may also include livedo
reticularis and Raynaud phenomenon
(due to the fact that this is a medium
vessel disease)
Significant weight loss
Men: testicular pain or tenderness
5. Mononeuropathy or polyneuropathy
6. Diastolic BP> 90 mmHg
7. Presence of HBsAg in serum
8. Arteriogram demonstrating aneurysms
or occlusions of visceral arteries
(transient loss of vision)
9. Biopsy of small or medium- sized artery
showing polymorphonuclear leukocytes
(PMNs) in wall
FOR CONCLUSIVE DIAGNOSIS, THE PATIENT
MUST HAVE BIOPSY!!
Angiogram will show multiple aneurysms*
CHANG-STRAUS SYNDROME Autoimmune, inflammatory systemic disease that is
uniquely associated with asthma and eosinophilia
Like Wegener’s granulomatosis:
Asthma
Recurrent sinusitis
Constitutional sx
Peripheral neuropathy
Mononeuritis multiplex
GI pain
ACR criteria: Patient must have 4 of 6 criteria
- Diagnosis (asthma)
- Eosinophilia (>10 % in peripheral blood)
Elevated Eosinophil count (>10
%)
Elevated ESR,
Elevated CRP
In the presence of clinical suspicion
Initial diagnostic step is a biopsy of an
affected area (skin, renal, muscle, lung,
nerve)
Prednisone alone is sufficient, unless the
symptoms are life threatening
*Higher rate of coronary involvement (aneurysms)*
WAY TO REMEMBER THIS DISEASE LOL:
*If Wegener’s disease and Polyarteritis Nodosa got
together and had a bastard child, it’s name will be
Chung- Strauss, and he’d have asthma*

25. - Has chronic sinus and nose involvement (usually
doesn’t perforate though)
- Involves the small vessels
- Can cause glomerulonephritis (UA=RBCs++)
- Tends to be ANCA positive (but it’s p-ANCA)
- Granulomatous on biopsy
Like Polyarteritis Nodosa :
- Has neuromuscular involvement
- Can develop mononeuritis multiplex, peripheral
neuropathies
- Postprandial abdominal pain is a common
complaint
Unique to Chang- Straus :
- Patient ALWAYS has asthma or some kind of
reactive airway syndrome
- Patient has eosinophilia (>10 %)
Cough
Arthralgias
Hemoptysis (remember, PAN spares the
lungs >_< )
Hematuria
Purpuric rash
- Paranasal sinusitis
- Mononeuritis multiplex or
polyneuropathy
- Pulmonary infiltrates (on CXR or CT)
- Histological proof of vasculitis
w/extravascular eosinophils
ACD present
UA will generally be abnormal
showing a GMN picture
(RBCs++, RBC casts)
p- ANCA+
Chung- Straus shares features of both Wegener’s
disease and Polyarteritis Nodosa and the patient with Chung- Strauss always
has asthma

26. - Biopsy shows eosinophils in granuloma
TEMPORAL ARTERITIS (GIANT
CELL ARTERITIS)
Inflammatory disease of the vessels involving the
medium and large arteries of the head/scalp (branches
of the ECA, most prominently the superficial temporal
artery)
Occurs always only in patient over the age of 55
Painful temples
Pain on chewing
Tender scalp
Visual disturbances:
- Diplopia
- Blurry vision
May co-exist with polymyalgia
rheumatica (proximal muscle pain)
PX:
- Scalp tenderness
- Superficial temporal artery is
visibly inflamed/engorged on the
temple or lateral forehead
N/A The best initial diagnostic step is to get an
erythrocyte sedimentation rate (ESR)
Most accurate test: temporal artery biopsy
*If the ESR is elevated, proceed straight to
treatment (Prednisone)*
You must also get a temporal artery biopsy
for confirmation, as it is the most common
test.
Prednisone

27. TAKAYASU’s ARTERITIS
(PULSELESS DISEASE)
Inflammatory disease of the LARGE VESSELS!!
( implicating the aorta and usually its initial ascending
branches (L Brachiocephalica, R Common Carotid , R
Subclavian a), however, it may descend all the way to
the aortic bifurcation)
Most commonly affects women of childbearing age (
<40 years).
More prevalent in Asians
Variable and non- specific Headache
Malaise
Arthralgia
Visual deficits.
Claudication of the extremities
Stoke
TIA and seizures are rare
manifestations
PX:
- Very important for establishing
suspicion
- Carotid or subclavian bruit
- Aortic regurgitation
- Weak upper extremity pulse
- BP difference between arms of
Diagnosis is likely if pt has 3 of the 6 criteria :
1. Younger than age 40
2. Extremity claudication
3. Decreased pulsation of one or both
brachial arteries
4. Difference in systolic BP of >10 mmHg
between arms
5. Subclavian bruit or abdominal aorta bruit
6. Arteriographic evidence
The most accurate test is arteriography Prednisone is the first line of therapy
Cyclophosphamide or Methotrexate may be added if there is no significant response to
prednisone alone
*Very important to ensure normal BP and manage
other cardiovascular risk factors, especially in
pregnancy*

28. >10 mmHg
BEÇHET’s SYNDROME Inflammatory, multisystemic disease of SMALL
VESSELS!!
resulting in frequent aneurysm and rupture
Idiopathic
Affects:
- Eyes
- Mucous membranes
- Genitals
*recurrent aphthous ulcers*
Recurrent aphthous ulcers
Painful genital ulcers
Eye pain w/redness (uveitis)
Rash (erythema nodosum acne like
rash)
Rarer but more serious symptom
include:
Meningoencephalitis
GI bleed
Amyloidosis
ISG Criteria for Behcet’s Syndrome:
*MUST HAVE*
- Recurrent oral aphthous ulceration (3x
in 12 month period)
* AND 2 OF THE FOLLOWING*
- Recurrent genital ulceration or scarring
- Eye lesions:
- Anterior uveitis
- Posterior uveitis,
- Retinal vasculitis (dx by ophto)
- Skin lesions:
- Erythema nodosum
- Acneiform nodules
- Papulo-pustular lesions (in patient
who has no other explanation)
BASED ON SYMPTOMS!!!!!
ORAL ULCERS:
(Topical corticosteroids, colchicine)
GENITAL ULCERS:
(Topical corticosteroids)
UVEITIS:
(Topical corticosteroids)
RETINAL VASCULITIS:
Cyclosporine

29. - Positive patherge test:
- Insert hypodermic needle into
skin, after 48 hours, papule present
in the region
KAWASAKI DISEASE Is also known as mucocutaneous lymph node
Syndrome
Is an acute self limited febrile illness of infants and
children (< 5 yrs)
JAPAN, HAWAII ( ENDEMIC)
KD is a childhood vasculitis that mainly targets
coronary arteries
Coronary artery involvement: – can lead to coronary
thrombosis or aneurysm formation and its rupture
Features:
Oral Erythema
Palmar Erythema
Conjunctivitis
Rash
Desquamation
Edema: FEET & ARMS
Clinical Findings:
- High fever
– Erythematous rash of trunk and
extremities with desquamation of skin.
N/A Neutrophilic leukocytosis
Thrombocytosis :
* characteristic finding*
Elevated ESR
Abnormal ECG
*acute MI*

30. – Mucosal inflammation : cracked lips,
oral erythema
– Erythema, swelling of hands and feet.
– Localized lymphadenopathy (cervical
adenopathy)
– MCC of an acute MI in children****
BUERGER’s DISEASE Also known as Thromboangitis Obliterans.
Is a peripheral vascular disease of smokers.
Pathology:
Earliest change:
- Acute inflammation involving the small to
medium sized arteries in the extremities (tibial,
popliteal & radial arteries).
Inflammation of vessel:
- Thrombus formation
Clinical Findings:
- Young-middle age, male, heavy
smoker ( Is***l, Japan, India)
Symptoms start between 25 to 40 years:
- Early manifestation:
- Intermittent Claudication
in feet or hands.
(Cramping pain in muscles
after exercise, relieved by
rest)
- Late manifestation:
N/A BIOPSY Early stages of vasculitis frequently cease on discontinuation of smoking.

31. - Obliterates lumen
- Ischemia
- Gangrene of extremity
Inflammation also extends to adjacent veins and nerves.
(Involvement of entire neurovascular compartment)
- Painful ulcerations of
digits
- Gangrene of the digits
often requiring
amputation.
HENOCH SCHONLEIN PURPURA
(HSP)
A variant of hypersensitivity vasculitis.
Seen in children (MC vasculitis in children)
Rare in adults.
Usually occurs following an upper respiratory infection
Caused by deposition of IgA-C3 immune complexes in
vessel wall.
Vessels involved:
- Arterioles
Clinically characterized by: –
- Palpable purpura over extensor
aspects of arms and legs.
- commonly limited to
lower extremities/
buttocks.
Involvement of:
● GIT
○ colicky abdominal pain,
melena
● Musculoskeletal system:
N/A
Neutrophilic leukocytosis
Deposition of IgA-C3 immune
complexes in:
Skin
Renal lesions
STEROIDS

32. - Capillaries and venules of:
- Skin
- GIT
- Kidney
- Musculoskeletal system.
○ Arthralgia (non migratory)
○ Myalgias
● Kidneys:
○ hematuria due to focal
proliferative GN
● Lung:
○ Rare
INFECTIOUS VASCULITIS Fungal vasculitis: vessel invading fungi
- Mucor
- Aspergillus
- Candida
Rocky Mountain spotted fever:
- Rickettsia
Disseminated meningococcemia:
- Small vessel vasculitis
N/A

33. - petechial hemorrhages
Infective endocarditis:
- Roth’s spots in retina
- Janeway’s lesions on hands (painless)
- Osler’s nodes on hands (painful)
- Glomerulonephritis
SJÖGREN’s SYNDROME Autoimmune, inflammatory and destructive disease of
the exocrine organs (primarily parotid glands, lacrimal
ducts, etc)
Affects 0.1- 4 % of the US population
Female is preponderance
Primary and secondary forms
History of numerous dental caries, due to
chronically dry mouth
In many cases, the patients will have a history of
another autoimmune disease (SLE, RA, etc) as
many as 1/3 of RA patients also have Sjogren’s
syndrome
The major symptoms are:
- Dry eyes (xerophthalmia)
- Dry mouth (xerostomia)
- Parotid gland enlargement
Typical presentation :
- dry eyes / dry mouth in female
patients
Pts must have 2 of the following 3:
Ocular staining score at least 3
Biopsy of salivary gland (single most accurate
test) showing lymphocytic sialadenitis
Positive ANA . Specifically Anti-Ro and Anti-La
CRITERIA BASED
*Biopsy of salivary gland (single most
accurate test*
Schirmer’s test:
- This test consists of placing a small
Lymphoma is a serious complication of SS, occurring LATE in the disease.
Risk of lymphoma is 44 times greater than the general population
20-30% of patients have vasculitis.
Renal involvement in 10% of patients interstitial nephritis

34. Complications:
- NonHodgkin’s lymphoma
- Neonatal lupus
- Some degree of parotid gland
swelling may be present
Mild to moderate sensorineural hearing
loss in high frequencies.
*Anti-cardiolipin antibodies has been
demonstrated, but no cochlear or
vestibular pathology has been found*
Nodes:
- Granulomatous and
non-granulomatous laryngeal
nodes
- Bamboo nodes - whitish or
yellowish transverse submucosal
lesions localized to middle third
of vocal fold.
Rare first symptom:
- Hoarseness
Lacrimal-gland biopsy
- Focal lymphocytic sialadenitis in minor
salivary glands containing more than 50
lymphocytes per 4 mm of glandular tissue
strip of filter paper inside the lower
eyelid (conjunctiva sac).
- The eyes are closed for 5 minutes.
- The paper is then removed and the
amount of moisture is measured.
- A young person normally moistens 15
mm of each paper strip.
- Because hyperlacrimation occurs
with aging, 33% of normal elderly
persons may wet only 10 mm in 5
minutes
- Persons with Sjögren's syndrome
moisten less than 5 mm in 5 minutes
RESULTS OF SCHIRMER TEST!!!!
1. Normal:
- which is ≥15 mm wetting of the paper
after 5 minutes.
Renal tubular acidosis also seen

35. Thyroid abnormalities:
- “Hashimotos’s thyroiditis” can
be found in up to 1/2 of Sjögren’s
syndrome patients
- 10-15% of Primary Sjögren’s
syndrome are clinically
hypothyroid
- Therefore, all patients with this
syndrome should undergo
routine evaluation of thyroid
function
50% having nasal mucosa hypertrophy
13% have septal perforations.
Pulmonary Symptoms:
- Xerotrachea (dry cough)
- Dyspnea
2. Mild:
- which is 14-9 mm wetting of the
paper after 5 minutes
3. Moderate:
- which is 8-4 mm wetting of the paper
after 5 minutes.
4. Severe:
- which is < 4 mm wetting of the paper
after 5 minutes.

36. Esophageal spasm and dysmotility are
also seen, but may be secondary to
absence of saliva,
- Atrophic gastritis
- celiac disease
GOUT -- Crystal deposition in the joint---
- inflammation, pain
-- Acute pain attacks
-- Men affected more than women
-- Blacks more than whites.
-- Average age of initial onset is 30-60 years
*Most often in early patients*
-- Urate crystal deposition in the joint, resulting in
tends to occur in older men( RF= age):
increased risk in:
- Overweight
- Alcohol drinkers
- Patients on diuretics (loop ( furosemide)
and thiazide)
Excruciating monoarticular joint pain
*may wake up the patient*
Overlying erythema
visible swelling
occasionally there may be nodules in
the soft tissue.
*Inflamed joints*
Fever may be present if there is
“polyarticular” involvement
N/A The best initial diagnostic step is: synovial
fluid analysis (arthrocentesis)
*don’t need to do every time*
Maybe diagnosing clinically if the patient
has had several confirmed attacks of gout
and this episode is similar
The best initial step in therapy is NSAIDs
*We treat the pain first !*
To prevent further gout attacks, patients should be started on:
- Allopurinol:
- Usual dose is 300 mg/day. The Maximum recommended dose is 800 mg/day.
- Dose depends on Kidney function:
- GFR
- Check creatinine
- In renal insufficiency dose should be decreased to 200 mg/day for
creatinine clearance < 60ml/min and to 100 mg/day if clearance < 30
ml/min).
- Start with small doses of allopurinol to reduce the risk of precipitating an
acute gout attack.
- Most common side effects are:
Maybe mistaken for cellulitis (over the affected joints)
Hallmark: Only in Gout Patients

37. inflammation and pain.
-- Most commonly first occurs in the big toe
(significant pain will occur too)
● May also occur in the:
○ Ankle
○ Knee
○ Proximal Interphalangeals (PIPs)
○ Distal Interphalangeals (DIPs)
○ Maybe polyarticular (fever also)
*monoarticular = no fever*
- Rash (2% of patients)
- rarely patients can develop exfoliative dermatitis that can be lethal
Colchicine may be used as well, but it has “significant GI effects”
Febuxostat is another, more recent drug on the market
Patients with multiple gout attacks should undergo:
- A 24- hour uric acid collection
*Do not fall for the trap of ordering serum uric acid levels !!! They don’t
correspond to gout attacks!*

38. PSEUDOGOUT Deposition of calcium pyrophosphate dehydrate,
calcium oxalate or calcium hydroxyapatite crystals in
the joint, resulting inflammation and pain
Tends to occur in older patients with pre-
existing joint disease and in patients with
metabolic or electrolyte disorders (particularly
involving calcium) *Hypercalcemia,
Hypophosphatemia, Hyperparathyroidism,
Hemochromatosis, Hypomagnesemia*
Presentation is almost identical to gout
not GOAT ( lol >_<)
Most common joint affected is the
knee.
The wrist, ankle, and shoulder may also
get affected
N/A Uric Acid: normal values
ranges:
- from 4.0 to 8.6 mg/dl in
men
- from 3.0 to 5.9 mg/dl in
women.
Urinary levels are normal
below 750 mg/24h
Urinary levels above 750 mg/dl
in 24h in gout or > 1100 mg/dl
in asymptomatic
hyperuricemia indicates urate
overproduction
The best diagnostic test is: Synovial fluid
analysis (arthrocentesis)
Like gout, the best initial therapy is NSAIDs
*Consider workup for underlying cause*
If Comprehensive Metabolic Panel (CMP) shows elevated calcium levels, consider getting a
PTH level
May put patient on low dose colchicine as a prophylactic for future attacks.
Diet is usually impractical, ineffective and rarely adhered to in clinical practice ( for
both )
SCLERODERMA
(CREST SYNDROME & SYSTEMIC
SCLEROSIS)
Scleroderma is a symptom (thickening of the
skin)
A term that can refer to two similar but separate
clinical syndromes
Almost all (more than 90%) of patients
with scleroderma also have Raynaud's
phenomenon.
Systemic Sclerosis:
ACR criteria for Systemic sclerosis
Patients must have one major criteria or two
minor criteria
CREST SYNDROME:
Clinical, major differential is systemic
sclerosis
Systemic Sclerosis:
- Symptomatic.
Major focus is in preventing scleroderma renal crisis (malignant
hypertension) and minimizing pulmonary hypertension
Pulmonary manifestations of systemic sclerosis occur in more than 70% of

39. Systemic sclerosis *aka diffuse systemic sclerosis* →
CREST + MULTI ORGAN
Systemic sclerosis and CREST syndrome both involve
cutaneous symptoms (skin thickening, calcinosis,
Raynaud syndrome, histological changes)
“Systemic Sclerosis” can affect the:
- Kidneys (renal insufficiency)
- The heart (CHF)
- Lungs (pulmonary fibrosis)
*CREST SYNDROME DOES NOT AFFECT*
Systemic sclerosis is:
- CREST syndrome PLUS Multiple internal organ
involvement
Idiopathic and Female predominance
Can have many different presentations
CREST syndrome *aka limited systemic sclerosis,
cutaneous sclerosis*
- Generally have symptoms similar
to CREST syndrome
- is not limited to peripheral
extremities and often affects the
face and neck.
- Other non- specific symptoms
may be present:
- Palpitations
- Shortness of breath
- Fatigue
- Weakness
Major criteria:
- Proximal scleroderma
Minor criteria:
- Sclerodactyly
- Digital pitting scars or loss of substance
from finger pad
- Basilar pulmonary fibrosis
Patients with CREST syndrome should have
titers ran for ANA (90% of patients will be+)
and anti- Scl- 70
Systemic Sclerosis:
Clinical, supported with laboratory evidence
Patients + for Anti- Scl-70 are more likely to
have diffuse systemic sclerosis
Treating CREST and Systemic sclerosis:
- Focused on symptoms and in systemic sclerosis, preventing scleroderma renal
crisis, and limiting any other complications
- Skin thickening : D-penicillamine
- Raynaud attacks: Calcium channel blockers (nifedipine) *sometimes a-blockers &
Nitrates*
- Scleroderma renal crisis (ACE inhibitors)
- GI symptoms: Proton- pump inhibitors manage closely for Barrett’s esophagus
PULMONARY:
- Most common SSc-related cause of death.
- Pulmonary diffuse fibrosis is a common cause of severe restrictive lung disease
- CXR shows interstitial thickening, most easily seen in the lower lung fields.
- ECG→ Right ventricular hypertrophy
*Some patients with interstitial lung
disease develop slowly progressive
patients
In Diffuse systemic sclerosis:
- Interstitial lung disease (ILD) more common
In Limited systemic sclerosis:
- Pulmonary hypertension is more common
The pulmonic component of the second heart sound is accentuated, and
ultimately, right-sided cardiac failure develops.
Diagnosis is confirmed by:
- echocardiogram or by right heart catheterization.

40. res
Sig
(PA
15%
cat
RE
CA

41. *Ac
Car
Sec
Or
MU

42. Mo
Als
NE