Progressive systemic sclerosis in Egyptian patients: Clinical characteristics and nail fold capillaroscopy

Aim of the work

To present the clinical and laboratory disease characteristics in Egyptian patients with progressive systemic sclerosis (SSc) and their nail fold capillaroscopy findings.

Patients and methods

50 SSc patients were included. For all patients detailed history taking and clinical and rheumatological examination were performed. The modified Rodnan skin score (mRSS) and nail fold capillaroscopy were assessed.

Results

The mean age was 41.4?±?12.7?years (range 16–67?years) and median disease duration was 5?years (range 1–40?years). Eight patients (16%) were males and 42 (84%) females (M:F 1:5.25). Skin tightness was present in all patients; mean mRSS was 17.5?±?10.4 (ranges 4–45). 44 (88%) patients had abnormal pulmonary function tests; 42 (84%) restrictive and 2 (4%) obstructive; 25 (50%) presented with interstitial lung disease. Pulmonary artery hypertension was recorded in 9 (18%) patients. Renal manifestations were the least common recorded system involvement (20%). Antinuclear antibodies were positive in 98%, rheumatoid factor in 4%, antitopoisomerase I in 36% and anticentromere in 8% of patients. 96% of patient had abnormal nail fold capillaroscopy. There was a significant negative correlation between mRSS with forced expiratory volume (r?=??0.46, p?=?0.001), forced volume capacity (r?=??0.47, p?=?0.001) and positively with the FEV1/FVC (r?=?0.3, p?=?0.035).

Conclusion

Almost all patients have ANA seropositivity, abnormal PFTs and abnormal nailfold capillaroscopy. Antitopoisomerase I antibody seropositivity, ILD, abnormal PFT, worsening skin score, late pattern of nailfold capillaroscopy are more common in dcSSc. Extensive clinical examination and investigations should be considered for early diagnosis and follow up of Egyptian SSc patients.