A Multidisciplinary Approach as a Goal for the Management of Complications in Systemic Scleroderma: A Literature Review and Case Scenario

• Published in: Diagnostics (Basel) Vol. 13; no. 21; p. 3332
• Main Authors: Pătrîntașu, Dariana-Elena, Sárközi, Hédi Katalin, Lupușor, Eugeniu, Vlangăr, Irina Elena, Rotariu, Gheorghe-Marian, Rența, Ionuț-Alexandru, Nan, Anda-Nicoleta, Budin, Corina Eugenia
• Format: Journal Article
• Language: English
• Published: Basel MDPI AG 01.10.2023
• Subjects: Adipocytes; Cytokines; Dyspnea; Esophagus; Fecal incontinence; Fibroblasts; Fingers & toes; Health aspects; Hypertension; Immune system; Inflammation; interstitial lung disease; Literature reviews; Lung diseases; Mortality; Musculoskeletal system; Pathophysiology; Patients; Pulmonary arteries; Pulmonary fibrosis; Pulmonary hypertension; Raynaud’s syndrome; Romania; Scleroderma; Scleroderma (Disease); Skin; Systemic scleroderma; systemic sclerosis; Ulcers; Romania
• ISSN: 2075-4418; 2075-4418
• DOI: 10.3390/diagnostics13213332

Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement. Scleroderma has the highest mortality among all rheumatic diseases. The pathophysiology mechanism of systemic sclerosis is a progressive self-amplifying process, which involves widespread microvascular damage, followed by a dysregulation of innate and adaptive immunity and inflammation and diffuse fibrosis of the skin and visceral organs. Fibrosis of internal organs is a hint for systemic sclerosis, moreover associated with interstitial lung disease (SSc-ILD) is a complex process. In order to correlate scientific data from the literature with clinical experience, we present the case of a 56-year-old woman who was diagnosed with systemic sclerosis 16 years ago. The association of numerous comorbidities characterized by a considerable level of seriousness characterizes this case: the highly extensive systemic damage, the cardiovascular impact of the illness, and the existence of severe pulmonary arterial hypertension. The systemic and clinical manifestations, respiratory functional tests, radiological features, and specific therapy are discussed.