Systemic Sclerosis(scleroderma)

Question 1

Diffuse cutaneous systemic sclerosis

Answer 1

• skin thickening proximal to the elbows and knees. May affect face
• ANA and anti-Scl-70 ab
• interstitial lung dz
• scleroderma renal crisis is present
• CREST syndrome absent

Question 2

Limited cutaneous systemic sclerosis

Answer 2

• skin thickening distal to elbows and knees, possibly affecting the face
• ANA and anticentromere ab positive
• Pulm HTN
• no scleroderma renal crisis
• CREST syndrome present

Question 3

Scleroderma renal crisis

Answer 3

• features include: HTN, microangiopathy, hemolytic anemia, thrombocytopenia, proteinuria, nonoliguric kidney failure
• steroid therapy is a risk factor assoc with normotensive renal crisis

Question 4

Scleroderma mimics

Answer 4

Eosinophilic fasciitis
Mixed connective tissue disease
Idiopathic Pulm fibrosis

Question 5

Eosinophilic fasciitis

Answer 5

Woody induration of the skin
Spares hands and feet
Peripheral blood eosinophilia
Diagnosis by full thickness skin biopsy

Question 6

Mixed connective tissue disease

Answer 6

Inflammatory myopathy
SLE features
Arthritis 
Scleroderma 
Anti-RNP ab

Question 7

Management of scleroderma

Answer 7

• CCB, SILDENAFIL, NITRO PASTE for raynaud
• PPI for GERD
• pro motility agents for dysmotility
• ACEi for scleroderma renal crisis regardless of serum Cr even in setting of kidney failure
• NO steroids

Question 8

Diagnostic criteria

Answer 8

Must have one or more of the following:

• sclerodactyly
• digital pitting
• interstitial lung disease
• raynaud phenomenon
• Pulm HTN
• polyarticular arthritis
• GERD
• pseudo-obstruction(GI-small bowel)
• malabsorption due to bacterial overgrowth