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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

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Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
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[Systemic sclerosis : A disease with multiple faces].

Caroline Evers1, Jörg Distler2, Oliver Distler3

  • 1Klinik für Rheumatologie, Universitätsspital Zürich, Rämistr. 100, 8091, Zürich, Schweiz.

Innere Medizin (Heidelberg, Germany)
|December 1, 2025
PubMed
Summary
This summary is machine-generated.

Systemic sclerosis (SSc) is a rare autoimmune disease affecting skin and organs. Understanding its diverse manifestations and tailoring treatments are key for effective patient care.

Keywords:
Antifibrotic therapyConnective tissue diseasesImmunosuppression therapyRaynaud diseaseSkin fibrosis

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Area of Science:

  • Rheumatology and immunology
  • Connective tissue diseases
  • Autoimmune disorders

Background:

  • Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease.
  • Characterized by vasculopathy and inflammatory fibrosis affecting skin and internal organs.
  • Presents with heterogeneous manifestations complicating diagnosis, monitoring, and treatment.

Purpose of the Study:

  • To provide an overview of diverse systemic sclerosis manifestations.
  • To summarize current treatment approaches for SSc.
  • To emphasize the importance of individualized treatment strategies.

Main Methods:

  • Literature review of recent advances in SSc research.
  • Synthesis of information on disease manifestations.
  • Compilation of current therapeutic options.

Main Results:

  • SSc exhibits a wide range of clinical presentations.
  • Therapeutic options for SSc have expanded.
  • Individualized treatment based on specific manifestations is crucial.

Conclusions:

  • Optimal patient care requires a multidisciplinary approach involving various specialists.
  • Close collaboration among rheumatologists, pneumologists, gastroenterologists, cardiologists, nephrologists, and general practitioners is essential.
  • Tailored treatment strategies are vital for managing SSc effectively.