Rheumatology

Question 1

[Rhem/JIA]
Juvenile idiopathic arthritis definition?

1. Occurring before < __ years of age
2. _ in ≥ 1 joints
3. _ for ≥ 6 weeks

Answer 1

1. Occurring before < 16 years of age
2. Persistent synovitis in ≥ 1 joints
3. Synovitis for ≥ 6 weeks

Question 2

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis and usual ANA finding?

Between 1 and 5 years old, M=F
Daily high fever ≥ 102.2F for ≥ 2 weeks, resolves without antipyretics (quotidian fever), usually in the evening
Salmon colored rash, appeared by mild rubbing (Koebner phenomenon)
Arthritis (usually polyarticular)
Pericarditis, myocarditis, pleuritis, lymphadenopathy, hepatosplenomegaly, abdominal pain
Leukemoid reaction (WBC > 40K), thrombocytosis (> 1 mil)

Answer 2

Systemic JIA

ANA: Negative

Question 3

[Rhem/JIA/Cx]
Which disease? (Complications from sJIA)

Persistent fever
Hepatosplenomegaly
Markedly high ferritin
Cytopenias
Liver dysfunction
Neurologic dysfunction

Answer 3

Macrophage activation syndrome

Question 4

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≤ 4 in the first 6 months of disease
Between 1 and 3 years old, F>M
Asymptomatic anterior uveitis
Non-specific laboratory findings (+/- ANA, +/- Inflammatory markers)

Answer 4

Oligoarticular JIA

Question 5

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≥ 5 in the first 6 months
Between 1-3 and 9-14, F>M
ANA (+)
Rheumatoid factor (-)
Less aggressive symptoms

Answer 5

RF Negative polyarticular JIA

Question 6

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≥ 5 in the first 6 months
Teenage, F>M
ANA (-)
Rheumatoid factor (+)
Anti-CCP ab (+)

Answer 6

RF Positive polyarticular JIA

Question 7

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Arthritis (DIP joint)
Psoriasis or Familial history of psoriasis
Dactylitis
Nail finding: pitting, onycholysis
HLA-B27 (adolescent with back pain)

Answer 7

Juvenile psoriatic arthritis

Question 8

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Teenage male
Arthritis
Enthesitis, Sacroiliac joint tenderness
Asymptomatic uveitis or irititis
Acute symptomatic uveitis
HLA-B27 (+), ANA (-), ESR normal

Answer 8

Enthesitis-related arthropathies

Question 9

[Rhem/JIA/Tx]
Treatment options for JIA (3)?

Answer 9

1. NSAIDs (initial treatment)
2. DMARDs - Methotrexate (with folate)
3. Joint steroid injections (2nd line)

Question 10

[Rhem/JIA/Tx]
Side effect of methotrexate (usual treatment for JIA) (3)?

Answer 10

1. Liver abnormalities
2. Oral ulcers
3. Cytopenias

Question 11

[Rhem/Arthritis with IBD]
Characteristics of Peripheral arthritis associated Inflammatory Bowel Disease

• Incidence F _ M
• HLA-B27 association: _
• Arthritis flares with gut flares: _

Answer 11

Peripheral arthritis

• Incidence F = M
• HLA-B27 association: no
• Arthritis flares with gut flares: yes

Question 12

[Rhem/Arthritis with IBD]
Characteristics of Axial arthritis associated Inflammatory Bowel Disease

• Incidence F _ M
• HLA-B27 association: _
• Arthritis flares with gut flares: _

Answer 12

Axial arthritis

• Incidence F < M
• HLA-B27 association: yes
• Arthritis flares with gut flares: no

Question 13

[Rhem/Arthritis]
Which arthritis and treatment?

1-4 weeks after GI or GU infection
Sterile pyuria, dysuria (urethritis)
Conjunctivitis
Arthritis, enthesitis, dactylitis
Oral, genital ulcers, balanitis
Keratoderma blenorrhagicum (papular skin lesions)
HLA-B27 (+), ANA (-), RF (-)

Answer 13

Reactive arthritis
(can’t pee, can’t see, can’t bend knee)

• Treatment:
  NSAIDs for 2-4 weeks

Question 14

[Rhem/Vasc/Sm]
Examples of small vessel vasculitis? (4)

Answer 14

1. Drug reations
2. Serum sickness
3. IgA vasculitis (HSP)
4. Granulomatosis with polyangitis (GPA)

Question 15

[Rhem/Vasc/Med]
Examples of Medium vessel vasculitis? (2)

Answer 15

1. Polyarteritis nodosa (PAN)
2. Kawasaki disease (KD)

Question 16

[Rhem/Vasc/Larg]
Examples of Large vessel vasculitis? (1)

Answer 16

Takayasu arteritis

Question 17

[Rhem/Vasc/Sm]
Symptoms of IgA vasculitis (HSP)?

A: __
A: __
R: __
P: __
P: __

Answer 17

Arthritis
Adominal pain
Renal disease
Palpable
Purpura

Question 18

[Rhem/Vasc/Sm]
Other manifestations of IgA vasculitis (HSP)?
(Other than purpura, abdominal pain, renal disease)

GI: __
GU: __
Pulm: __

Answer 18

GI: Intussusception
GU: Orchitis
Pulm: pulmonary hemorrhage

Question 19

[Rhem/Vasc/Sm]
Laboratory findings of IgA vasculitis (HSP)?

ESR: __
IgA: __
Platelet: __
PT/PTT: __

Answer 19

ESR: elevated
IgA: elevated
Platelet: normal
PT/PTT: normal

Question 20

[Rhem/Vasc/Sm]
Which syndrome?

Glomerulonephritis
Purpura
Arthritis
Pulmonary hemorrhage
Saddle nose
Ocular: conjunctivitis, dacryocystitis, scleritis, proptosis

Anti-proteinase-3 antibodies
c-ANCA positive (specific for proteinase 3 (PR3))

Answer 20

Granulomatosis with Polyangiitis (GPA): pulmonary renal syndrome

GN
Pulmonary
Arthritis

If p-ANCA: Microscopic Polyangiitis

Question 21

[Rhem/Vasc/Med/KD]
Diagnostic criteria for Kawasaki Disease?

Fever ≥ __ days
C: __
R: __
E: __
A: __
M: __

Answer 21

Fever ≥ 5 days
+
4 of 5 followings:
Conjunctival injection without drainage
Rash: polymorphous exanthema, later desquamation
Edema: hands/feet
Adenopathy: cervical, unilateral, >1.5 cm
Mucosal involvement: strawberry tongue, cracked lips

Question 22

[Rhem/Vasc/Med/KD]
Treatment options for Kawasaki Disease?

Answer 22

Aspirin 80-100 mg/kg/day

AND

IVIG 2 g/kg

Question 23

[Rhem/Vasc/Med]
Which vasculitis?

M>F around 9 years old
Skin nodules
Abdominal pain
Hypertension
Mononeuritis multiplex (foot drop)
Kidney injury, orchitis
HBV infection, strep infection, certain drugs
(No lung involvement)

Answer 23

Polyarteritis nodosa (PAN)

Paralysis: foot drop
Abdominal pain
Nodules, skin
(No Pulm)

Question 24

[Rhem/Vasc/Larg]
Which vasculitis?

Coarctation of aorta, HTN
Fever, arthritis
Claudication, decreased peripheral pulses

Answer 24

Takayasu arteritis

Question 25

[Rhem/JSS] Which disease and antibodies? **C**alcinosis **R**aynaud phenomenon **E**sophageal dysmotility **S**clerodactyly **T**elangiectasia

Answer 25

Limited cutaneous scleroderma - Antibodies: anticentromere antibody (diffuse: anti-SCL 70 antibody)

Question 26

[Rhem] Which disease? Lung: hilar infiltrates, restrictive lung PFT Hepatosplenomegaly, mediastinal lymphadenopathy Erythema nodosum Eye: uveitis, conjunctival granulomas

Answer 26

Sarcoidosis

Question 27

[Rhem/Periodic Fever] Diagnosis, cause and treatment? Armenians, Turks, Levantine Arabs Fever with predictable patterns Abdominal pain Erysipelas like rash around the ankle Arthritis Pleuritis, pericarditis, scrotal swelling

Answer 27

Familial mediterranean fever - Cause: MEFV gene - pyrin defect - Treatment: Colchicine

Question 28

[Rhem/Periodic Fever] Diagnosis, cause and treatment? Dutch, French Fever for 3-7 days every 1-2 months Abdominal pain Large-joint arthritis Erythematous macular rash Lymphadenopathy Oral/vaginal ulcers Elevated IgD

Answer 28

Hyper IgD syndrome - Cause: AR, MVK gene - mevalonate kinase defect

Question 29

Pfapa treatment

Question 30

Rheuma toid condicionó + sensor heaving loss Muckle-wells Il-1 nhibitors

Answer 30

This child has Muckle-Wells syndrome, a rare genetic disorder that is one of the cryopyrin-associated periodic syndromes (CAPS). This form is characterized by recurrent fevers, urticaria, and joint pain. Characteristically, sensorineural hearing loss tends to develop in later years or adolescence, followed by progressive renal failure due to amyloidosis arising from long-standing inflammation. Cryopyrin is involved with the activation of interleukin (IL)-1β. Targeted inhibition of the IL-1 pathway has resulted in new and better treatments for these diseases. FDA-approved drugs include the IL-1 inhibitors rilonacept, anakinra, and canakinumab.

Question 31

Bechet

Answer 31

It is also associated with several systemic manifestations—including genital ulcers, uveitis, a variety of cutaneous lesions, arthritis, gastrointestinal ulcerations, and vasculitis of any size artery or vein—affecting the central nervous system (CNS), kidney, and/or heart. Anterior uveitis presents as erythema of the conjunctiva, which is most prominent at the junction of the sclera and cornea (the limbus); pupillary constriction; photophobia; and disturbance in vision. In order to meet the diagnostic criteria for Behçet disease, outbreaks of oral ulcerations must occur at least 3× a year. Healing is typically spontaneous within 1–3 weeks, although ulcers continue to persist in some patients. Genital ulcers are similar in appearance to oral lesions and are most common on the scrotum in males or the vulva in females. Associated cutaneous lesions include erythema nodosum, acneiform lesions, erythema multiforme—like lesions, and papulovesicular or pustular eruptions. Asymmetric nondeforming arthritis, typically affecting the medium and large joints, is particularly common during exacerbations of the disease.

Question 32

Antibodies and asociase síndrome Ana dsdna Smith s Le → dermato Aldo Ccp, rf ra