Systemic Sclerosis

Question 0

Subset of systemic sclerosis patients who are more likely to develop interstitial lung disease

Answer 0

Diffuse cutaneous systemic sclerosis (dcSSc)

Question 1

Subset of systemic sclerosis patients who are more likely to develop pulmonary arterial hypertension in the absence of other pulmonary manifestations

Answer 1

Limited cutaneous systemic sclerosis (lcSSc)

Question 2

In the absence of skin changes proximal to the MCP, diagnosis of systemic sclerosis may also be established in patients manifesting two of these 3 signs

Answer 2

Sclerodactyly, terminal digital pitting or ulceration, or basilar interstitial fibrosis on CXR

Question 3

Recommended drug in systemic sclerosis to prevent recurrences of severe digital ulcers

Answer 3

Endothelin receptor antagonist (bosentan)

Question 4

Most common gastrointestinal manifestation of systemic sclerosis

Answer 4

Esophageal dysfunction

Question 5

Confirmatory test for bacterial overgrowth syndrome in the setting of bowel dysmotility in systemic sclerosis

Answer 5

Glucose hydrogen breath testing

Question 6

Subset of systemic sclerosis patients who are more likely to develop primary biliary cirrhosis

Answer 6

Localized cutaneous systemic sclerosis (lcSSc)

Question 7

In systemic sclerosis, patients with elevated titers of this antibody have the highest risk for developing interstitial lung disease

Answer 7

Anti-Scl-70

Question 8

Best imaging for interstitial lung disease in systemic sclerosis

Answer 8

High-resolution noncontrast CT

Question 9

Screening test for PAH in systemic sclerosis

Answer 9

Pulmonary function test

Question 10

A localized form of scleroderma presenting as one or more indurated plaques confined to the torso and proximal extremities

Answer 10

Morphea

Question 11

Scleredema with large, noninflammatory indurated plaques over the shoulder girdle, neck, and upper extremities occurs as a complication of this condition

Answer 11

Insulin-dependent diabetes mellitus

Question 12

Waxy yellow-red papules overlying thickened skin; due to deposition of mucin in the dermis; frequently associated with paraproteinemia (most commonly IgG-lambda) in the setting of multiple myeloma or AL amyloidosis

Answer 12

Scleromyxedema

Question 13

Eosinophilic fasciitis presents as woody induration of the extremities and spares these body parts (2)

Answer 13

Hands (digits) and face

Question 14

Treatment for scleroderma renal crisis during pregnancy

Answer 14

ACE inhibitors