Chapter 44 - Systemic Sclerosis (Scleroderma) and Related Disorders Flashcards Preview

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Flashcards in Chapter 44 - Systemic Sclerosis (Scleroderma) and Related Disorders Deck (13):

What's the first-line treatment for eosinophilic fasciitis?

Oral corticosteroids
*A response to treatment is seen within a few weeks, and the dose of prednisone is tapered over 6-24 months, as tolerated


Can eosinophilic fasciitis (Shulman's syndrome) be diagnosed by MRI?

Yes; it can also be diagnosed by biopsy
*Prompt treatment is required to preserve mobility and function


True or false: eosinophilic fasciitis (Shulman's syndrome) usually affects the hands, feet, and face.

False; it typically spares the hands, feet, and face


What class of drugs are the treatment of choice for esophageal dysmotility in patients with scleroderma?

Proton pump inhibitors


Why should a patient with scleroderma have their blood pressure monitored?

Because they may have renal involvement, leading to renal crisis. If the systolic pressure goes up 20 mmHg, or if the diastolic pressure goes up 10 mmHg, an ACE inhibitor should be started


What are the different types of cutaneous fibrosis?

Systemic sclerosis/scleroderma, morphea/localized morphea, eosinophilic fasciitis, scleredema, scleromyxedema, nephrogenic systemic fibrosis


What are the subtypes of systemic sclerosis (SSc)?

Limited, diffuse


What are the subtypes of morphea/localized scleroderma?

Plaque, linear, generalized


What are the subtypes of scleredema?

Type I/post-infectious, type II/monoclonal gammopathy-associated, type III/diabetes mellitus associated


What is the single major criteria (as per ACR) in the diagnosis of SSc?

Symmetric cutaneous sclerosis proximal to the MCP or MTP joints (you need major + 2 minor)


What are the 3 minor criteria in the diagnosis of SSc?

1) Sclerodactyly, 2) digital pitting scars or loss of substance from the finger pad, 3) bibasilar pulmonary (you need major + 2 minor)


What are the 3 phases of SSc?

1) edematous, 2) indurated, 3) atrophic and +/- 4) recovery phase. There can also be associated dyspigmentation, telangiectasia, calcinosis cutis, Raynaud's phenomenon, ulceration


What is the treatment for SSc?

No cure. Symptomatic: 1) Raynaud's - avoid cold, tobacco, vasodilators, 2) ulcers - wound care, 3) skin - nothing, ?D-penicillamine, 4) calcinosis cutis - warfarin, 5) kidney - ACEi, 6) GI - PPI, prokinetic, 7) lung - cyclophosphamide, oral dual endothelin receptor antagonist