Characteristics of Progressive Systemic Sclerosis in a Cohort of Egyptian Patients

Mahmoud A,Abugabal M,Abdelzaher A,Alhassanein Kf,Soliman H,Alhefny A,Haroon Mm,Sayed S,Abdelmoteleb S

doi:10.21767/1989-5216.1000256

Abstract

Objective: To estimate the frequency of epidemiological, clinical and laboratory characteristics of progressive systemic sclerosis in a cohort of Egyptian patients. Methods: Fifty systemic sclerosis patients were included. These patients were subjected to detailed history taking, clinical and rheumatological examination, Measuring the dermal skin thickness by the modified Rodnan skin score (mRSS), Nail fold capillaroscpy (NFC) and the relevant radiological, laboratory and immunological investigations. Results: Our results revealed that the mean age at time of diagnosis was 32.66 ± 13.08 with the disease durations range from 1 to 40 years with a median of five years. Male to female ratio of 1: 5.2 and 20% of patients were smokers. Skin tightness was present in all patients, the mRSS ranges from 4 to 45 with a mean of 17.48 ± 10.44. ANA was detected in 98%, RF was detected 4%, antitopoisomerase I (antitopo I) was detected in 36% and ACA was detected in 8% of patients. 96% of patient had abnormal NFC. There were statistical significant negative correlations between mRSS and both of FEV1% and FVC%. There was also a statistical significant positive correlation between mRSS and FEV1/FVC. Conclusion: This study has shown that almost our Egyptian SSC patients have ANA seropositivity, abnormal pulmonary function tests and abnormal nailfold capillaroscopy (NFC). The study revealed that anti topo I antibody seropositivity, ILD, abnormal pulmonary function tests, worsening skin score, late pattern of NFC are more common in Diffuse Cutaneous Systemic Sclerosis (DCSSC) than Limited Cutaneous Systemic Sclerosis (LCSSC). Also ILD in SSC patients is commonly associated with antitopo I antibody seropositivity, abnormal pulmonary function tests, worsening skin score and late pattern of NFC. Therefore ANA, antitopo I, high resolution CT chest, pulmonary function test, mRSS and NFC should be considered for early diagnosis and follow up of SSC patients.

Highlights

Systemic sclerosis (SSC) is a rare multisystemic connective tissue disease characterised by microvascular damage, fibrosis of the skin and internal organs and specific immunologic abnormalities
Systemic sclerosis is an autoimmune disease in which fibrosis of the skin and internal organs occur in association with small vessel vasculopathy and autoantibody production
This is a cross-sectional cohort study in which 50 adult patients, diagnosed with progressive systemic sclerosis according to the 2013 American College of Rheumatology/ European League Against Rheumatism Systemic Sclerosis classification criteria [7], were recruited from the outpatient clinic and inpatient departments of rheumatology in Ain Shams university and military hospitals in the period between November 2014 to September 2016

Summary

Introduction

Systemic sclerosis (SSC) is a rare multisystemic connective tissue disease characterised by microvascular damage, fibrosis of the skin and internal organs and specific immunologic abnormalities. The clinical recognisable disease is classified on the basis of extent of skin involvement into subsets with diffuse cutaneous involvement (DCSSC) and limited cutaneous involvement (LCSSC) [1]. Systemic sclerosis (scleroderma, SSC) is an autoimmune disease in which fibrosis of the skin and internal organs occur in association with small vessel vasculopathy and autoantibody production. Organ-specific and non-organ specific impairments lead to a spectrum of mild to severe limitations in physical, work and social activities, influencing health-related quality of life [2]. As with many other autoimmune disorders, scleroderma is approximately 4–5 times more common in women than men. The average age at the time of diagnosis is approximately 50 years [3]

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