Systemic Sclerosis: Tools for Diagnosis and Treatment

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Hematology and Immunology".

Deadline for manuscript submissions: closed (31 August 2021) | Viewed by 7864

Special Issue Editor


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Guest Editor
Department of Rheumatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400006 Cluj, Romania
Interests: connective tissue diseases—systemic sclerosis; systemic lupus erythematosus; dermato-/polymyositis; Sjogren syndrome; rare connective tissue diseases; biologic therapies; medical education; art and medicine

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc) is a complex and rare autoimmune disease which carries the highest mortality and morbidity within the spectrum of connective tissue diseases. SSc involves almost every organ system in the body. Symptoms vary greatly among patients and can dramatically impact one’s life. The prevalence of SSc may be underestimated. Cases are often undiagnosed or misdiagnosed. Due to late referral to rheumatologic care, many moderate-to-severe cases progress to irreversible end-organ damage which might be prevented by early diagnosis. The recently improved understanding of SSc pathogenesis, which is a combination between fibrosis (explaining the skin, gastrointestinal, lung, and cardiac involvement), vasculopathy (responsible for Raynaud’s phenomenon, renal crisis, pulmonary arterial hypertension, and macrovascular disease), and inflammation (explaining the autoantibodies and the cardiac, muscular or articular involvement), has expanded the array of diagnostic tools. The progress also allows for better care, including a new set of classification criteria, more assessment and follow-up tools, as well as advances in treatment. There are now several effective drugs and updated evidence-based recommendations to manage many of the different organ-based manifestations. Treatment strategies combine the use of immune suppression (including autologous haemopoietic stem cell transplantation), antifibrotics, and vasoactive drugs. This Special Issue offers the clinician an updated review of the tools for diagnosis and treatment in SSc.

Prof. Simona Rednic
Guest Editor

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Keywords

  • Systemic sclerosis
  • Vasculopathy
  • Fibrosis
  • Inflammation
  • Autoantibodies
  • Pulmonary arterial hypertension (PAH)
  • Interstitial lung disease (ILD)
  • Gastroesophageal reflux
  • Malabsorption
  • Antifibrotic therapies
  • Immunosuppression

Published Papers (1 paper)

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Review

15 pages, 4064 KiB  
Review
Clinical and Histopathological Features of Scleroderma-like Disorders: An Update
by Rosario Foti, Rocco De Pasquale, Ylenia Dal Bosco, Elisa Visalli, Giorgio Amato, Pietro Gangemi, Riccardo Foti and Alice Ramondetta
Medicina 2021, 57(11), 1275; https://doi.org/10.3390/medicina57111275 - 20 Nov 2021
Cited by 8 | Viewed by 7448
Abstract
Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical–pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud’s phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis [...] Read more.
Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical–pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud’s phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis with systemic sclerosis. When scleroderma can be excluded, on the basis of the main body sites, clinical evolution, any associated pathological conditions and specific histological features, it is possible to make a correct diagnosis. Full article
(This article belongs to the Special Issue Systemic Sclerosis: Tools for Diagnosis and Treatment)
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