Rheumatology

Question 1

Systemic IgA-mediated vasculitis involving the joints, GI tract, and kidneys

Answer 1

Henoch-Schönlein Purpura

Question 2

Who does HSP typically affect?

Answer 2

Children younger than 10 y/o (median age of onset = 5 years)

Males more likely affected (M-F ratio = 2:1)

Question 3

What are the clinical features of HSP?

Answer 3

1. Viral syndrome or URI precedes HSP in most patients (20% of pts have concomitant or prior group A beta-hemolytic streptococcal infection)
2. Skin, GI, joint, renal manifestations follow prodrome
   
   1. ​Skin:
      
      1. Urticarial or erythematous maculopapular lesions –> petechiae and palpable purpuric lesions concentrated on butt and lower extremities
      2. Edema of hands, feet, scrotum, scalp
   2. Joint:
      
      1. 80% of patients: Arthralgia or arthritis (knees and ankles most commonly involved)
   3. GI:
      
      1. 67% of patients: colicky abdominal pain, GI bleed
      2. Inc. risk of intussusception
   4. ​Renal:
      
      1. Wide range of presentation - mild hematuria to gross hematuria, nephrotic syndrome, chronic renal insufficiency, ESRD (1% of cases)
      2. Renal manifestations may not ecome clinically apparent for up to 3 mo after initial presentation in 25% of pts who develop nephritis

Question 4

How to dx HSP?

Answer 4

1. Inc. serum IgA levels present in 50% pts
2. Circulating IgA immune complexes in serum and IgA deposition in skin and glomeruli
3. Platelet counts are normal despite presence of petechiae and purpura (i.e., the skin rash is a nonthrombocytopenic purpura)

Question 5

Mgmt/prognosis of HSP?

Answer 5

Treatment based on relief of symptoms (pain control/hydration)

• Steroids may be effective for relief of abdominal pain/arthritis

Prognosis:

1. Most pts recover within 4 weeks
2. HSP recurs at least once in 50% of pts
3. Long-term morbidity is dependent on severity of nephritis

Question 6

Acute febrile vasculitis of childhood, of unknown origin involving multiple organ systems, including the heart, skin, mucous membranes, GI tract, CNS, joints, and peripheral vascular bed

Answer 6

Kawasaki Disease (mucocutaneous lymph node syndrome)

Question 7

Diagnostic Criteria of Kawasaki Disease (5)

Answer 7

Dx of exclusion… must r/o infectious causes, rheumatologic conditions (JRA), drug rxns

Then:

1. Fever > 102 lasting more at least 5 days
2. 4 of the 5 criteria must be met
   
   1. Conjunctivitis-b/l (w/o exudate)
   2. Rash (primarily on trunk… erythematous maculopapular)
   3. Adenopathy (cervical)
   4. Strawberry tongue (oropharyngeal changes: pharyngitis, red, cracked, swollen lips)
   5. Hands and feet (edema early… peeling around nail beds late)

Question 8

Most serious complication of Kawasaki disease

Answer 8

Coronary artery aneurysms (occur in 20% of untreated Kawasaki usually around days 7-14)

Question 9

What is the time course and associated laboratory findings of Kawasaki Disease?

Answer 9

Triphasic:

1. Phase I: acute phase (1-2 weeks)
   
   1. ESR, CRP
2. Phase II: subacute phase (weeks to months)
   
   1. Platelet count, decreasing ESR and CRP
3. Phase III: convalescent phase (weeks to yrs)
   
   1. Lab values usually normalize around 6-8 weeks

Question 10

Kawasaki Disease: mgmt

Answer 10

IVIG & high dose aspirin

Question 11

Distinguishing Features of Juvenile Rheumatoid Arthritis Subtypes

Answer 11

Question 12

Rare type of inflammatory arthritis that is similar to RA; shares characteristics of juvenile idiopathic arthritis but it begins in adulthood

Answer 12

Systemic-onset JRA (Still’s Disease)

Question 13

Symptoms of Still’s Disease

Answer 13

1. High spiking fevers (temperatures > 102.2) occur most commonly in the late afternoon or evening and subsequenlty return to baseline or subnormal levels
   
   1. Typically included in the differential dx of “fever of unknown origin”
2. Transient salmon-colored rash most commonly found on trunk & proximal extremities, especially during febrile episodes
   
   1. Rash = evanescent (occurs with fever spikes and then fades) and nonpruritic
3. Hepatosplenomegaly
4. Lymphadenopathy
5. Other features:
   
   1. Fatigue, anorexia, weight loss, FTT
   2. Serositis
   3. CNS involvement (meningitis, encephalopathy)
   4. Myositis, tenosynovitis

Question 14

Diagnostic Criteria for SLE*

*4 of 11 criteria provide sensitivity and specificity of 96%

Answer 14

Question 15

This inflammatory condition of muscle results in progressive muscle weakness with characteristic skin findings.

Answer 15

Dermatomyositis

Question 16

Characteristic cutaneous findings

Dermatomyositis

Answer 16

• Periorbital violaceous heliotrope rash
• Gottron’s papules

Question 17

Main treatment for dermatomyositis

Answer 17

Corticosteroids

Question 18

Association with malignancy in pediatric dermatomyositis?

Answer 18

No

(In adult disease, malignancy develops in 25% of pts)

Question 19

Major risk factor for Group A beta-hemolytic strep?

Answer 19

Pharyngitis

(Streptococcal strains that cause skin infection i.e., impetigo, do NOT cause rheumatic fever)

Question 20

Laboratory findings in JIA

Answer 20

• Elevated inflammatory markers (ESR, CRP)
• Hyperferritinemia
• Hypergammaglobulinemia
• Thrombocytosis
• Anemia (due to inc. production of hepcidin which inhibits iron absorption across the small intestine and iron release from macrophages and reticuloendothelial system of the liver)

Question 21

How to confirm dx of B burgdorferi?

Answer 21

ELISA & Western Blot

Question 22

ANA

Answer 22

Sensitive Lupus

Question 23

Histone

Answer 23

Specific Drug-Induced Lupus

Question 24

ds-DNA

Answer 24

Specific Lupus + Renal Involvement

Question 25

Smooth Muscle

Answer 25

AI Hepatitis

Question 26

Centromere

Answer 26

Scleroderma (CREST)

Question 27

RO+LA

Answer 27

Sjogren’s

Question 28

CCP

Answer 28

Rheumatoid Arthritis

Question 29

RF

Answer 29

Rheumatoid Arthritis

Question 30

Jo

Answer 30

Polymyositis

Question 31

Topoisomerase (Scl-70)

Answer 31

Systemic Scleroderma

Question 32

Rheumatoid Arthritis + Neutropenia + Splenomegaly

Answer 32

Felty’s Syndrome

Question 33

What type of x-ray would you want to get a pt with RA?

Answer 33

Cervical spine to examine C1 and C2

Question 34

RA: Treatment

Answer 34

Question 35

Urethritis + Arthritis + Uveitis

Answer 35

Reiter’s Syndrome

Treat: Doxycycline + NSAIDs

Question 36

Answer 36

Question 37

Answer 37