Today we discussed scleroderma (AKA systemic sclerosis)
This is a multisystem disesase of small vessels and skin/organ fibrosis
Epidemiology: Age 35-65, Female more common than male (7-12:1).
There are 2 types of systemic sclerosis- limited and diffuse
Limited SSc: sclerosis, thickening of distal limbs without truncal involvement. CREST fits into this category. Associated with anti-centromere AB. Calcinosis refers to localized hand masses on fingers/forearms. Telangiectasias are seen in face/mucosa/hands. Most serious manifestations of limited are pulm HTN, digital ischemia.
Diffuse SSc: thickening over distal and proximal limbs and trunk with significant organ involvement. Criteria are thickening of extremities proximal to MCPs or digital ischemia, pulmonary fibrosis. Edematous skin, painful joints, tendon friction rub, rapidly progressive skin fibrosis. Associated with SCL70 antibody.
Clinical:
Initially nonspecific; Raynaud's, fatigue, MSK complaints followed in variable time period by further sx (soon in diffuse, later in limited).
First specific manifestations are skin thickening, starting as swelling/puffiness over fingers/hands.
Subsequently variable; skin, pulmonary, cardiac, renal, GI involvement.
90% of SSc pts have Raynaud's and fibrosis of fingers, with loss of digital pads (sclerodactyly) and digital ulceration.
Skin: edema, tightness, decreased flexibility, contractures, then fibrosis.
MSK: arthralgias, arthritis, calcinosis, tendon involvement. Late: weakness/atrophy, possible overlap myosits
Pulm: Pulmonary fibrosis, PFTs show decreased volumes, diffusion impairment.
GI: small mouth, dysphagia, GERD/esophagitis. Loss of peristalsis
Cardiac: Myocardial fibrosis, myocarditis, diastolic dysfunction
Renal: Pathological change in most (small vessel vasculopathy); extreme is scleroderma renal crisis (acute kidney injury, with microangiopathic hemolytic anemia)
Therapy: No proven disease modifying agents; treatment is by organ system involved. In severe cases, immunosuppression may be used (e.g. steroids, methotrexate, cyclophosphamide)
Skin- key is moisturize, treat pruritus
Raynaud's- CCB, prazosin in severe
GI- treat GERD, prokinetics for paresis
Renal- ACE-I
Pulmonary- measures for pulm HTN
Links:
Click here for a recent NEJM review of scleroderma
Click here for a review from Rheumatology on scleroderma
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