Peripheral neuropathy in course of progressive systemic sclerosis

Abstract

Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue with involvement of the skin and other organs. The disease is characterized by an abnormal accumulation of collagen in all tissues and by microangiopathy. The involvement of the peripheral nervous system during PSS is very unusual and few cases are reported in the literature. A morphological study on the neuropathy associated with sclerodermia has been performed in rare cases. In this paper we demonstrate the role that the vascular lesions have in the pathogenesis of neuropathy during scleroderma. In particular, the primary role of the peripheral microangiopathy during PSS (observed in different clinical cases) is verified.

Acid clearance and oesophageal sensitivity in patients with progressive systemic sclerosis.

“…This is consistent with the positive correlation between the frequency and duration of gastro-oesophageal reflux symptoms and oesophageal acid exposure in dyspeptic patients with or without oesophagitis. 9 Our PSS patients reported reflux symptoms, however, only during a small fraction of the acid exposure time, as previously seen in patients with chest pain or gastro-oesophageal reflux.21 Symptoms of reflux therefore reflected poorly acid exposure times in patients with PSS as they do in patients with reflux and no connective tissue diseases. The discrepancy between the exposure time to acid and the extent of symptoms was striking in PSS, perhaps because of the prolonged oesophageal acid exposure in these patients, particularly during the supine period.…”

Carpal Tunnel Syndrome and Systemic Sclerosis

“…Pathogenesis of neurological involvement in sclero derma may result both from a primary injury of vasa nervo rum or from perineural inflammation [17]. These mech anisms could explain the diffuse sensory neuropathies encountered in scleroderma [7].…”

Progressive Systemic Sclerosis Associated with Multiple Mononeuropathy

“…Other cases have been reported: PSS with polymono neuropathy; a few cases of trigeminal neuropathy [7][8][9], 2 cases of multiple mononeuritis in the limbs [10], 4 cases of peripheral neuropathy in the arms and legs [11], and 1 case of polyneuropathy in the legs [12], Trigeminal neuritis was not observed in either of our cases, but there was some truncal mononeuritis in case 1. There have been no other reports of truncal mononeuritis in PSS.…”

“…Involvement of the peripheral nervous system in the course of PSS is rare and very few cases have been reported [7][8][9][10][11][12], The peripheral neuropathy in our cases was char acterized by acute onset of multiple mononeuropathy with sensory impairment for all modalities associated with a tin gling sensation and well-preserved motor nerves. In con trast to the other clinical skin and serological signs of PSS, peripheral neuropathy occurred in both cases 1 year after the onset of PSS.…”