AB0924 EVALUATION OF PERIPHERAL NERVOUS SYSTEM INVOLVEMENT IN PATIENTS WITH JUVENILE SYSTEMIC SCLEROSIS AND JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

Background Juvenile systemic sclerosis (JSS) and juvenile systemic lupus erythematosus (JSLE) are rare connective tissue disorder characterized by multisystemic involvement, including gastrointestinal, cardiovascular, respiratory and nervous system complications. According to data from literature, peripheric nervous system (PNS) involvement is seen in 10-86.7% of adult patients with systemic sclerosis. Frequency of PNS disorders is reported as 10-86.7% in adult patients. Data on PNS involvement in patients with JSS and JSLE are scarce. Objectives We aimed to evaluate PNS involvement in patients with JSS and JSLE. Consequently, we sought to detect patients with PNS disorders, in order to enable early diagnosis and timely intervention. Methods Patients with JSS and JSLE were included in a cross-sectional study. Demographic and clinical data of patients were recorded during clinical visits. All of patients were evaluated and examined for sings of PNS involvement. In order to examine mononeuropathy, polyneuropathy and trigemino-facial involvement, all patients underwent routine nerve conduction studies (NCS), blink reflex (BR) and sympathetic skin responses (SSR) evaluation. Results Twenty JSS (15 (75%) female) and 18 (15 (83%) female) JSLE patients were initially included. All of JSS and JSLE patients had normal neurologic examination. NCS was normal in all JSS (20/20) and JSLE (18/18) patients. SSR was not recorded in 1 (5%) JSS and in 3 (16.67%) JSLE patients. BR was recorded in all JSLE (18/18) patients and in majority of JSS patients (19/20, 95%). According to SSR, mean latency of hand and foot was similar in both patients’ groups. Amplitude of foot response was lower in JSS patients, comparing to JSLE (Table 1). Among JSLE patients, amplitude of hand response was lower than amplitudes of foot response. According to BR, R1, R2, R2K duration and latency were not different between right and left eyes in both patients’ groups. R3 was absent in 2 (10%) bilaterally and in 1 (5%) JSS patients unilaterally (right). R3 was absent in 4 (22.2%) bilaterally and in 2 (11%) JSLE patients unilaterally. Absence of R3 was more prominenet in JSLE patients, comparing to JSS. (Table 2) Conclusion BR could be considered as a potential indicator of neuropathy in JSLE and JSS patients. Data on SSR need to be evaluated in studies with higher number of patients with juvenile-onset connective tissue disorders.