A clinical case of late diagnosis of systemic scleroderma

Abstract

Background. Systemic scleroderma (SSD), a chronic rheumatological disease of connective tissue of autoimmune origin, the pathogenesis of which is based on a chronic inflammatory process, severe microcirculation disorders, the development of systemic fibrosis, has in the expanded stage of the clinical picture a lesion of the skin, musculoskeletal, musculoskeletal system and almost all internal organs. It is very important that there is a holistic perception of scleroderma as one of the systemic diseases of connective tissue, which, with the limited therapeutic potential of drugs, has a tendency to an unfavorable prognosis due to the high probability of irreversible damage to internal organs. The purpose of the study is to analyse the polysyndromicity and severity of clinical manifestations of SSD, the need for a thorough comprehensive assessment of symptoms and syndromes, a wide range of diagnostic measures and the importance of timely appointment of adequate therapy. Materials and methods. The analysis of a clinical case (a patient aged 74 years who was treated in June 2019 in the Department of therapy No. 1 of the State Medical Institution “Penza Clinical Hospital No. 4”) is presented. Results. In this patient, who has typical signs of sclerodermic lesions of the skin and internal organs, a reliable diagnosis of systemic connective tissue disease was determined at the stage of virtually total, irreversible lesions. The effectiveness of therapy, despite the slight positive dynamics, was extremely insufficient, the phenomena of congestive pneumonia were stopped, but signs of severe heart failure remained almost unchanged, which predetermined an extremely serious prognosis not only for recovery, but also for life. Conclusions. Extremely late and untimely diagnosis of SSD is associated with long-term long-term observation and treatment of the patient by various narrow specialists, and each of them has its own separate disease. The above clinical case demonstrates the low awareness of primary care physicians about the clinical picture of systemic rheumatological diseases. It seems necessary, with the undoubted consideration of the modern development of the instrumental and laboratory base for the diagnosis of rare diseases, a wider introduction into clinical practice of diagnostic criteria for early diagnosis, as well as greater importance to attach to the development of a doctor's ability to systemically analyze complaints, anamnesis and physical examination of patients.

Keywords

• systemic scleroderma
• raynaud's syndrome
• chronic heart failure
• clinical case