Frequency of disease subsets and patterns of organ involvement among Egyptian scleroderma patients

Research Abstract

Summary Systemic Sclerosis (SSc) is a generalized disorder of connective tissue which is characterized clinically by skin thickening and fibrosis and by distinctive forms of internal organ involvement, especially, lungs, heart, kidneys and gastrointestinal tract. The etiology is unknown and females are usually more commonly affected than males by this disease. SSc is divided into two main subsets, according to the extent of skin affection in addition to the specific autoantibodies. These subsets are the limited cutaneous systemic sclerosis (lcSSc) and the diffuse cutaneous systemic sclerosis (dcSSc). This disease may lead to functional disability of the patients in various life aspects, and may also lead to shortened life expectancy especially in the case of pulmonary or renal affection. This work aims at determining the disease patterns and manifestations of organ involvement among scleroderma patients presented to our department. This study included 75 patients with systemic sclerosis diagnosed according to the ACR criteria (ARA, 1980). Their ages ranged between 17 and 70 years. The results of this study are the following: • Systemic sclerosis is more common in females than in males, with male : female ratio of 1 : 4.3. • Limited cutaneous systemic sclerosis (lcSSc) is more common than diffuse cutaneous systemic sclerosis (dcSSc) where the limited : diffuse ratio was 4.3:1. • Females were found to be more common than males within both disease subsets and the difference was statistically significant. • Raynaud's phenomenon was found to be the first presenting manifestation in 77.3% of patients, and it was found to be the commonest first presenting manifestation among both males and females, and also among the limited and the diffuse subsets. • The frequencies of organs involvement among patients were as the following: Raynaud's in 97.3%, digital ischemia in74.6%, dysphagia in 68%, interstitial lung disease (ILD) in 53.3%, arthritis in 40%, esophageal reflux in 38.7%, hypopigmentation was present in 22.7%, myositis in 20%, pulmonary HTN (P HTN) in 14.7%, dysmotility in 10.7%, proteinuria in 6.7% and pericardial effusion in 5.3% of the patients. • Interstitial lung disease, secondary pulmonary hypertension, proteinuria and intestinal dysmotility were found to be significantly more common among patients with dcSSc than patients with lcSSc. • Manifestations of organs involvement occurred with different frequencies between males and females but this difference wasn't statistically significant. Conclusion From this study we can conclude the following: *Systemic sclerosis is more common in females than in males. *Limited cutaneous systemic sclerosis is more common than diffuse cutaneous systemic sclerosis. *Raynaud’s phenomenon is the most common first presenting disease manifestation. *Diffuse cutaneous systemic sclerosis is more commonly associated with interstitial lung disease, secondary pulmonary hypertension, proteinuria and intestinal dysmotility than limited cutaneous systemic sclerosis. *Gender does not affect the frequency of organ involvement in a significant way. Recommendations 1- It is recommended to follow up closely and monitor any early diagnosed cases of diffuse cutaneous systemic sclerosis for the development of interstitial lung disease, pulmonary hypertension and protienuria because this group is more liable to develop these disease manifestations more than the limited group. 2- Further studies are recommended on larger groups of systemic sclerosis patients from multiple centers to establish the disease patterns and prevalence of disease manifestations among different disease subsets. 3- These data should facilitate research regarding the role of geographic, ethnic, racial, and environmental factors for this disease in comparing populations.

Research Keywords

Frequency of disease subsets and patterns of organ involvement among Egyptian scleroderma patients