Frequency of disease subsets and patterns of organ involvement among Egyptian patients with systemic sclerosis – A retrospective study

Abstract

Aim of the work

To determine the prevalence and disease characteristics of Systemic Sclerosis among Egyptian patients presented to Rheumatology departments in Cairo University & Bani Swief University.

Patients & methods

This is a retrospective study that included 75 patients with systemic sclerosis diagnosed according to the American college of rheumatology criteria. Their ages ranged between 17 and 70 years, all patients data were collected by medical records review.

Results

Out of the studied 75 patients, 14 (18.7%) were males and 61 (81.3%) were females with a ratio of 1:4.3. Fourteen patients (18.7%) had diffuse cutaneous systemic sclerosis and 61/75 (81.3%) had limited cutaneous systemic sclerosis. The commonest initial presenting manifestation in all patients was Raynaud’s phenomenon in 58/75 patients (77.3%), followed by arthritis in 9/75 patients (12%) & skin tightness in 7/75 patients (9.3%). Interstitial lung disease was significantly more frequent within the diffuse compared to the limited subtype (78.6%, 47.5%, respectively P = 0.042). Secondary pulmonary hypertension was significantly more common among the patients with dcSSc compared to the limited subtype (35.7%, 6.6%, respectively P = 0.009). No significant difference was observed between males and females in the disease pattern.

Conclusion

Limited cutaneous systemic sclerosis is more common than diffuse ‘subtype’. Raynaud’s phenomenon is the commonest initial presentation. Diffuse subtype is more commonly associated with interstitial lung disease, secondary pulmonary hypertension and proteinuria than the limited subtype. Gender does not affect the frequency of organ involvement significantly.