Polyneuropathy in Progressive Systemic Sclerosis

ABSTRACT

Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue which affects the skin and other internal organs. Involvement of the peripheral nervous system is rare, although muscle abnormalities as a component of PSS are well recognized. 29-year-old (patient 1) and 64-year-old (patient 2) women had PSS for 3 and 5 years, respectively. They complained of having a tingling sensation in the legs. Examination showed reduced strength in the distal muscles, bilateral hypesthesia for pain and vibration below the knees and reduced or absent tendon reflexes in the lower limbs. Sensorimotor neuropathies were demonstrated by decreased motor conduction velocities and pro-longed distal sensory latencies. Sural nerves were of normal thickness. The density of myelinated fibers was not decreased and axonal degeneration was scanty or absent. Characteristic findings were intimal hypertrophy and perivas-cular inflammatory infiltrates in both patients. Inflammatory cell invasions in the peripheral nerve is extremely are in patients with PSS. It may be suggested that an inflammatory or immune mechanism is another contributing factor to polyneuropathy in PSS as well as microangiopathy.