Abstract

OBJECTIVES: To describe the frequency, extent, and nature of microvascular lesions in patients with scleroderma by means of capillaroscopy and capillaroscopically guided nail fold biopsy, and to determine the diagnostic value of the two methods and the pathophysiological significance of the lesions described. METHODS: A cohort study was made of 24 consecutive patients with scleroderma and 10 healthy controls, using standardised clinical, serological, capillaroscopic, and histological (nail fold biopsy) techniques. RESULTS: All patients with scleroderma had distinct lesions of the microvascular system. Capillaroscopy revealed more than 90% of the patients to have the typical scleroderma pattern. Histologically, these changes most frequently consisted of splitting of the basal lamina, broadening of the perivascular connective tissue, perivascular round cell infiltrations, and immunoglobulin deposits (each in 60-75% of the patients). Electron microscopy was the most sensitive method of histological examination, detecting abnormalities in 87.5% of patients; with light microscopy and immunohistochemical techniques, abnormalities were revealed less frequently (83.3% and 75%, respectively). In contrast, normal findings were observed in most of the healthy controls: capillaroscopy = 90%; histology = 80%. CONCLUSIONS: Microvascular lesions are a predominant feature in scleroderma and seem to have a central pathogenetic role in the disease. Capillaroscopy is able to identify this microangiopathy noninvasively, and capillaroscopically guided nail fold biopsy can detect the frequency and nature of the underlying ultrastructural changes. This may therefore be a useful tool in describing the pathogenetic role of the microvascular system in scleroderma.