RESUMO
BACKGROUND: Scleroderma or systemic sclerosis (SSc) is a rare autoimmune rheumatic connective tissue disease. The clinical picture is manifold and symptoms can vary greatly between different patients. All manifestations are possible ranging from isolated skin involvement up to systemic disease with multiple organ manifestations. Due to this inhomogeneous clinical picture, it often takes years until the correct diagnosis is made and adequate treatment is started. METHODS: Patients with the main or secondary diagnosis of systemic sclerosis (M34) between 2002 and 2017 were retrospectively recorded from the patient databases of the ACURA clinic for acute rheumatology in Bad Kreuznach and the data were evaluated. Of special interest were pulmonary parameters over the course of time. Furthermore, standardized questionnaires were distributed to general practitioners in Rhineland-Palatinate via the Association of Statutory Health Insurance Physicians as well as to patients admitted to the hospital (2016-2017). RESULTS: A total of 135 patients could be evaluated. For women the median age of onset was 52 years (interquartile range, IQR 44-64 years) and for men the median age of onset was 49 years (IQR 38-54 years). Lung involvement was detected in 54% of the cases. Including the individual time to diagnosis, there was a significant worsening of the diffusing capacity for carbon monoxide (73% vs. 56%, pâ¯= 0.046) between earlier (<4 months) and later (4-18 months) diagnoses, which also persisted in the follow-up (74% vs. 53%) despite adequate treatment. CONCLUSION: A rapid diagnosis within 3 months of the onset of Raynaud's phenomenon seems to play a key role in the preservation of lung function.
