Case Report: Limited systemic sclerosis in a 78-year-old woman
A 78-year-old woman presented with Raynaud’s phenomenon, skin thickening of both hands distal to the wrists, telangiectasia, microstomy and soft tissue calcifications. She has a history of reflux esophagitis treated with proton pump inhibitors for acid suppression.
Limited systemic scleroderma (SSc) mostly affects the skin of the face, neck and distal elbows and knees and late in the disease causes isolated pulmonary hypertension. CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is associated with limited scleroderma. The most frightened complication is pulmonary arterial hypertension (PAH) which is observed more often in patients with limited SSc than in diffuse one.
Its treatment still remains a challenge for rheumatologists. In this case the following symptomatic treatment was initiated: glucocorticoids in medium to low doses; calcium channel blockers; methotrexate 20 mg/weekly percutaneous. Later in the disease course bosentan was introduced due to pulmonary hypertension.
Diagnosis
The 2013 ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease [1].
It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc–related autoantibodies.
1. Hoogen, Frank, et al. "2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative." Arthritis & Rheumatism 65.11 (2013): 2737-2747.
Tags: SSc, scleroderma, calcinosis, telangiectasia