PubMed · 1751321
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1991. Pediatric and heritable disorders.. https://doi.org/10.1097/00002281-199110000-00020
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Neuropathies are common in patients with known or suspected connective tissue disease. A vasculitic mononeuropathy multiplex is often seen in patients initially presenting with polyarteritis nodosa or developing arteritis as a complication of rheumatoid arthritis. However, vasculitic neuropathy may become confluent and present as as distal symmetrical polyneuropathy or occur without systemic necrotizing vasculitis. Distal symmetrical polyneuropathies without associated vasculitis are also common in many connective tissue diseases. Compression neuropathies, especially carpal tunnel syndrome, occur with increased frequency in rheumatoid arthritis. Finally, certain neuropathies may be the major presenting feature of particular connective tissue diseases. For example, trigeminal neuropathy often heralds the onset of systemic sclerosis or mixed connective tissue disease, and sensory neuronopathy may be the initial presenting feature of Sjögren's syndrome.
We have established a new diagnostic method using a points system to evaluate patients with early scleroderma and those with scleroderma spectrum disorders (SSD). To examine the clinical usefulness of this method, it was applied to a total of 215 cases including 97 patients with scleroderma, 32 with SSD, 28 with presumed primary Raynaud's phenomenon (RP) and 58 with other connective tissue disorders (CTD). A total score was obtained for each patient as the sum of the following five factors: (1) extent of skin sclerosis (maximum, 10 points); (2) pulmonary changes (maximum, 4 points); (3) antinuclear antibodies (maximum, 5 points); (4) pattern of Raynaud's phenomenon (maximum, 3 points); and (5) nailfold bleeding (maximum, 2 points). Of the 97 scleroderma patients, 86 (89%) had 9 or more points, and of the 32 SSD patients, 28 (88%) had 5 to 8 points. In contrast, all patients with presumed primary RP and 54 of 58 (93%) patients with other CTD had 0 to 4 points. These data suggest that this diagnostic method is very useful not only for clinical evaluation of SSD, but also for the differentiation of scleroderma and SSD from other CTD and primary RP.