Chapter 44 - Systemic Sclerosis (Scleroderma) and Related Disorders

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Yes; it can also be diagnosed by biopsy

*Prompt treatment is required to preserve mobility and function

Question 3

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

Answer 3

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

Question 4

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

Answer 4

Proton pump inhibitors

Question 5

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

Answer 5

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

Question 6

What are the different types of cutaneous fibrosis?

Answer 6

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

Question 7

What are the subtypes of systemic sclerosis (SSc)?

Answer 7

Limited, diffuse

Question 8

What are the subtypes of morphea/localized scleroderma?

Answer 8

Plaque, linear, generalized

Question 9

What are the subtypes of scleredema?

Answer 9

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

Question 10

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

Answer 10

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

Question 11

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

Answer 11

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

Question 12

What are the 3 phases of SSc?

Answer 12

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

Question 13

What is the treatment for SSc?

Answer 13

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