Chatper 45 - Other Rheumatologic Diseases

Question 1

Granulomatous dermatitis presenting in the context of autoimmune disease occurs in which two major clinicopathologic patterns?

Answer 1

Interstitial granulomatous dermatitis (IGD) with arthritis and palisaded neutrophilic and granulomatous dermatitis (PNGD)
*These occur in patients with RA or other autoimmune disorders

Question 2

Is methotrexate an effective treatment for rheumatoid vasculitis?

Answer 2

No; consider intravenous methylprednisolone, followed by prednisone and cyclophosphamide

Question 3

involving medium and large vessels requires aggressive treatment as it can be rapidly progressive and life-threatening.

Answer 3

True

Question 4

How is Felty’s syndrome usually treated?

Answer 4

With G-CSF and/or splenectomy

Question 5

Sterile infiltration of the skin by neutrophils can occur in patients with rheumatoid arthritis. Which primary neutrophilic disorders are most common in these patients? List three.

Answer 5

Pyoderma gangrenosum, Sweet’s syndrome, and rheumatoid neutrophilic dermatitis

Question 6

What’s the eponymous name for the uncommon but severe subset of seropositive rheumatoid arthritis patients that develop granulocytopenia, splenomegaly, and therapy resistant leg ulcers?

Answer 6

Felty’s syndrome

*The leg ulcers may be secondary to pyoderma gangrenosum or a medium-sized vessel vasculitis

Question 7

What are the 3 types of juvenile idiopathic arthritis?

Answer 7

1) Systemic-onset/Still’s disease, 2) polyarticular 3) oligo/pauciarticular types

Question 8

What are the different types of hereditary periodic fever syndromes?

Answer 8

Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D syndrome (HIDS), TNF receptor associated periodic syndrome (TRAPS), cryopyrin associated periodic syndromes (CAPS)

Question 9

What is the treatment for SoJIA?

Answer 9

Depends on severity. 1) Mild - NSAIDs, +/- hydroxychloroquine, 2) moderate-severe - PO steroids +/- other such as MTX, biologics, thalidomide

Question 10

What is the treatment for adult onset Still’s disease?

Answer 10

Aspirin, NSAIDs, PO steroids, steroid sparing agents

Question 11

What are some possible causes of Sjogren’s syndrome?

Answer 11

Genetic (HLA-B8, -DR3, -DQ2 and -DRw52a), autoAbs (anti-fodrin, anti-SSA/Ro, anti-SSB/La), increase CD40/CD154, increase bcl-2/bcl-x, ?viral infections (HIV, Hep C, human T cell leukemia virus 1)

Question 12

What is the treatment for Sjogren’s syndrome?

Answer 12

Symptomatic. Artificial lubricants/ointments, humidifier, hydroxychloroquine, PO fluids, dental hygiene (immunosuppressive agents/biologics in vasculitis/systemic involvement)

Question 13

Other than rheumatoid nodules and vasculitis, what are some other clinical findings of RA?

Answer 13

Bywater’s lesions, Felty’s syndrome, pyogenic gangrenosum, Sweet’s syndrome, rheumatoid neutrophilic dermatitis, erythema elevatum diutinum