Flashcards in Chatper 45 - Other Rheumatologic Diseases Deck (13):
Granulomatous dermatitis presenting in the context of autoimmune disease occurs in which two major clinicopathologic patterns?
Interstitial granulomatous dermatitis (IGD) with arthritis and palisaded neutrophilic and granulomatous dermatitis (PNGD)
*These occur in patients with RA or other autoimmune disorders
Is methotrexate an effective treatment for rheumatoid vasculitis?
No; consider intravenous methylprednisolone, followed by prednisone and cyclophosphamide
involving medium and large vessels requires aggressive treatment as it can be rapidly progressive and life-threatening.
How is Felty's syndrome usually treated?
With G-CSF and/or splenectomy
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.
Pyoderma gangrenosum, Sweet's syndrome, and rheumatoid neutrophilic dermatitis
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?
*The leg ulcers may be secondary to pyoderma gangrenosum or a medium-sized vessel vasculitis
What are the 3 types of juvenile idiopathic arthritis?
1) Systemic-onset/Still's disease, 2) polyarticular 3) oligo/pauciarticular types
What are the different types of hereditary periodic fever syndromes?
Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D syndrome (HIDS), TNF receptor associated periodic syndrome (TRAPS), cryopyrin associated periodic syndromes (CAPS)
What is the treatment for SoJIA?
Depends on severity. 1) Mild - NSAIDs, +/- hydroxychloroquine, 2) moderate-severe - PO steroids +/- other such as MTX, biologics, thalidomide
What is the treatment for adult onset Still's disease?
Aspirin, NSAIDs, PO steroids, steroid sparing agents
What are some possible causes of Sjogren's syndrome?
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)
What is the treatment for Sjogren's syndrome?
Symptomatic. Artificial lubricants/ointments, humidifier, hydroxychloroquine, PO fluids, dental hygiene (immunosuppressive agents/biologics in vasculitis/systemic involvement)