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

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,...

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Published inDiagnostics (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
LanguageEnglish
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
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Abstract 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.
AbstractList 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.
Audience Academic
Author Lupușor, Eugeniu
Nan, Anda-Nicoleta
Budin, Corina Eugenia
Pătrîntașu, Dariana-Elena
Rotariu, Gheorghe-Marian
Rența, Ionuț-Alexandru
Sárközi, Hédi Katalin
Vlangăr, Irina Elena
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  fullname: Nan, Anda-Nicoleta
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  fullname: Budin, Corina Eugenia
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Snippet Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement. Scleroderma has the...
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SubjectTerms 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
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Title A Multidisciplinary Approach as a Goal for the Management of Complications in Systemic Scleroderma: A Literature Review and Case Scenario
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Volume 13
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