Prevalence and severity of complicated Raynaud's phenomenon in limited and diffuse systemic sclerosis: a multicenter...

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multiple internal organ involvement, vasculopathy, and fibrosis. Two major types are present, limited systemic sclerosis (lSSc) and diffuse systemic sclerosis (dSSc), according to the limit of skin fibrosis, with variability in internal organ involvement. Raynaud's phenomenon (RP) is almost always present in either type as a presenting feature; it may precede the onset by years. It affects the quality of life for the patient and has a variable range of complications as well, with the most severe being tissue gangrene and finger amputation. The aim of the study was to investigate the prevalence of RP complications and predictors of outcome in lSSc and dSSc.

Patients diagnosed with SSc were included in the study at 3 rheumatology centers in Iraq over a 3-year period. Data collection was conducted through questionnaires and interviews. All patients underwent clinical assessments to determine the presence or absence of RP complications, including pit scars, ulcers, ischemia, and amputated digits at the time of interview as well as the previous records. Subjects with concomitant autoimmune diseases were excluded from the study.

Of the 105 patients, 55 (52%) had dSSc, and 92% of those were female. The mean age was in the fourth decade. Digital complications were recorded. Ischemia was the most frequently observed complication in limited scleroderma, while amputation was the least common. These complications exhibited a significant association with the duration of the disease, with the highest prevalence occurring within the first 10 years of diagnosis. Cardiac complications were associated with RP. Notably, 27% of patients with dSSc had hypertension, while 64% of patients with limited scleroderma did not have cardiac complications.

Raynaud's phenomenon is a defining characteristic of both limited and diffuse scleroderma. It is particularly complex in the diffuse form, underscoring the necessity for aggressive treatment to prevent debilitating complications. It is crucial to educate patients about the significance of adhering to treatment regimens and cessation of smoking.