Rheumatology

Question 1

What is the definition of JIA?

Answer 1

Synovitis in at least one joint for at least 6 weeks before the age of 16 with exclusion of other diagnoses (infection, malignancy).

Question 2

What are the 7 JIA categories?

Answer 2

Systemic, oligo, poly RF-, poly RF+, Psoriatic, Enthesitis-related, undifferentiated.

Question 3

What are some key cytokine mediators of JIA?

Answer 3

TNF-a, IL-1, IL-6, prostaglandins, complements, proteases, etc.

Question 4

What cell types are responsible for JIA symptoms?

Answer 4

Presentation of antigen by APCs to T-cells with subsequent expansion of T- and B-cells.

Question 5

At what age does systemic JIA peak?

Answer 5

2 years

Question 6

What systemic symptoms may be exhibited in systemic JIA

Answer 6

Quotidian fever, migrating salmon colored rash, myalgia, pericarditis, myocarditis, pleuritis, hepatosplenomegaly, abdominal pain, lymphadenopathy, weight loss, fatigue.

Question 7

In which JIA category or categories is RF positive?

Answer 7

Only RF+ polyarticular

Question 8

In which JIA category or categories is ANA positive?

Answer 8

Typically only oligoarticular and RF- polyarticular.

Question 9

True or False: ANA is useful in the diagnosis of JIA.

Answer 9

False, but it can be useful as a prognostic factor (in particular, ANA+ increases risk of uveitis associated with JIA).

Question 10

Highly elevated ferritin in a patient with systemic JIA suggests what complication?

Answer 10

MAS.

Question 11

What is the treatment of MAS?

Answer 11

Dexamethasone may be sufficient, but if the patient is not clinically stable and/or deteriorating, the HLH-94 protocol including dexamethasone, etoposide, cyclosporine (watch for PRES–some omit cyclosporine), and intrathecal methotrexate (if CNS symptoms, in which case intrathecal dexamethasone may be added also) should be initiated. As opposed to HLH, treatment of MAS might also include anakinra, IVIG, or other immunomodulators.

Question 12

What are the clinical features of HLH/MAS?

Answer 12

Fever, splenomegaly, cytopenia of 2 lines, hypertriglyceridemia, low NK activity, hemophagocytosis on biopsy (of node, bone marrow, liver or spleen), elevated Ferritin, elevated soluble CD25. While at least 5 of 8 of these features are required for diagnosis, treatment should not be delayed for laboratory confirmation is suspicion is high and patient is deteriorating. Other features include liver dysfunction and neurologic dysfunction.

Question 13

What complication are patients with oligoarticular JIA at risk for even in the absence of joint symptoms?

Answer 13

Anterior uveitis with potential formation of iris synechiae.

Question 14

How often should slit lamp exams be performed for patients with JIA?

Answer 14

It depends on age of onset, duration of disease, and ANA, but as often as every 3 months if patient is <6 years of age, ANA+, and has had arthritis for less than 4 years. Older, lower risk children can have an exam every 12 months.

Question 15

What is nail pitting a symptom of (in the context of JIA)?

Answer 15

Juvenile Psoriatic Arthritis

Question 16

HLA-B27 positivity is a feature of which categories of JIA?

Answer 16

Enthesitis associated and, in older patients, psoriatic.

Question 17

Which forms of JIA are NOT more common in females than males?

Answer 17

Systemic (equal) and enthesitis associated (male predominant)

Question 18

Which categories of JIA have a single age peak in the teen-aged years?

Answer 18

RF+ polyarticular and enthesitis associated.

Question 19

Which categories of JIA have a single age peak in early childhood (1-3 years)?

Answer 19

Oligoarticular, and systemic

Question 20

Which categories of JIA have a dual age peak, with the first at 1-3 years, and the second later in childhood (10 years+)?

Answer 20

RF- polyarticular and psoriatic

Question 21

What are the mainstays of JIA treatment?

Answer 21

NSAIDs, intra-articular steroid, DMARDs (most commonly methotrexate), Biologics (if DMARDS fail), and systemic steroids (only for flares and resistant disease–try to minimize due to side effects).

Question 22

What are some TNF inhibitors in clinical use as biologic therapy for DMARD resistant JIA?

Answer 22

Etanercept, adalimumab, infliximab

Question 23

What is an IL-6 inhibitor in clinical use as biologic therapy for DMARD resistant JIA?

Answer 23

Tocilizumab

Question 24

What are some IL-1 inhibitors in clinical use as biologic therapy for DMARD resistant JIA?

Answer 24

Anakinra, canakinumb, rilonacept.

Question 25

What organisms are associated with reactive arthritis

Answer 25

Strep, but also GI bugs (Yersinia, shigella, salmonella, Giardia, C Diff, Campylobacter), Chlamydia, and Lyme disease.

Question 26

What is the clinical triad of reactive arthritis

Answer 26

Urethritis, conjunctivitis, arthritis

Question 27

What are some small vessel vasculitides of childhood?

Answer 27

HSP (IgAV), serum sickness, drug reactions, granolumatosis with polyangitis (GPA–formerly Wegener’s). (Note that anti-GBM disease, formerly Goodpasture’s, is similar to GPA but very, very, very rare in children, while GPA is only very very rare. Anti-GBM is characterized by pulmonary hemorrhage rather than granulomas.)

Question 28

What are two medium vessel vasculitides of childhood?

Answer 28

Kawasaki disease and polyarteritis nodosa

Question 29

Name a large vessel vasculitis

Answer 29

Takayasu arteritis

Question 30

HSP (IgAV) is preceded by what in 50% of cases?

Answer 30

Upper respiratory infection

Question 31

What is the formal definition of HSP (IgAV)

Answer 31

An immune-mediated leukocytoclastic vasculitis with neutrophil infiltration and primarily IgA deposition in vessel walls.

Question 32

What are the most common clinical features of HSP (IgAV), in decreasing order of prevalence?

Answer 32

Skin lesions, joint symptoms (arthritis/periarthritis), GI symptoms (pain, occult bleeding, rarely overt bleeding), IgA mediated nephropathy (almost always self limited), orchitis, pulmonary hemorrhage.

Question 33

What follow up is needed for patients after recovery from acute phase of HSP (IgAV)?

Answer 33

UA and blood pressure monitoring. Regimens vary, but here is one: weekly for two months, monthly for 6 months, every other month for 6 more months, then annually.

Question 34

True or False: NSAIDs for treatment of joint pain are contraindicated in HSP (IgAV).

Answer 34

False. Despite risk of GI tract bleeding in HSP, in the absence of active bleeding there is no demonstrable increase in risk with use of NSAIDs in this context.

Question 35

What special consideration is required when using steroids for HSP (IgAV)

Answer 35

A long taper is required to reduce risk of rebound symptoms (4-8 weeks).

Question 36

Name two contraindications to use of NSAIDs for treatment of joint and/or abdominal pain associated with HSP (IgAV)

Answer 36

Active GI bleeding or glomerulonephritis.

Question 37

A 9 year old patient presents with mononeuropathy, hypertension, and painful skin nodules. ANCA is negative. What is the diagnosis?

Answer 37

Polyarteritis nodosa

Question 38

A patient presents with gangrenous necrosis of a toe, skin ulcers, and bowel perforation, and neuropathy that began as mononeuropathy but is now a polyneuropathy. What is the diagnosis?

Answer 38

Advanced stage polyarteritis nodosa.

Question 39

What is the treatment for PAN?

Answer 39

Remission induction with steroids and cyclophosphamide (note that cyclophosphamide improves disease control but not survival). If related to Hepatitis B infection, antiviral therapy and plasma exchange are recommended in addition to steroids.

Question 40

Recurrent apthous ulcers (3 times in a year or more) should prompt consideration of what diagnosis?

Answer 40

Behçet disease.

Question 41

Behçet disease classically presents as oral mucosal ulceration, genital ulcers, and ocular inflammation. What other organ systems can be involved?

Answer 41

Just about any, in part because Behçet disease can lead to vasculitis with accompanying thrombosis. Examples include GI ulceration and bleeding mimicking (or possibly actually representing a variant of) Crohn’s disease. Arthritis is common. Less common are CNS involvement (both parenchymal and vascular), cardiac, and renal involvement.

Question 42

What is the treatment for Behçet disease?

Answer 42

Topical or systemic steroids. For significant end organ disease (GI, CNS, etc.) require immunomodulation both to achieve remission and limit need for systemic steroids. Azathioprine is commonly used, or TNF-alpha modulators in some contexts. (infliximab, etanercept). Colchicine is commonly used for arthritis, as well as renal disease due to amyloid deposition.