Related Experiment Videos

[Treatment of systemic sclerosis]

F H J van den Hoogen1

  • 1Department of Rheumatology, Radboud University Medical Centre Nijmegen & Rheumatology Centre St Maartenskliniek, PO Box 9101, 6500 HB , Nijmegen, Niederlande. F.vandenHoogen@reuma.umcn.nl

Zeitschrift Fur Rheumatologie
|July 5, 2006
PubMed

Insights

Systemic sclerosis treatments show modest benefits. Cyclophosphamide improves lung function in scleroderma patients with alveolitis, while other therapies stabilize pulmonary hypertension.

Area of Science:

  • Immunology
  • Rheumatology
  • Pulmonology

Context:

  • Systemic sclerosis (SSc), or scleroderma, is a severe multisystem autoimmune disease with limited curative treatment options.
  • Immune dysregulation is a key factor in SSc pathogenesis, leading to widespread use of immunosuppressive therapies.
  • SSc frequently involves critical organ systems, including the lungs, leading to significant morbidity and mortality.

Purpose:

  • To review current therapeutic strategies for systemic sclerosis.
  • To evaluate the efficacy of immunosuppressive agents and targeted therapies in managing SSc complications.
  • To highlight recent advancements in SSc treatment based on clinical trial data.

Summary:

  • Cyclophosphamide demonstrated a modest but significant improvement in pulmonary function tests for SSc patients with alveolitis in placebo-controlled trials.
  • Prostacyclin analogues and endothelin-1 receptor antagonists have shown efficacy in stabilizing pulmonary arterial hypertension, a life-threatening SSc complication.
  • Current treatments aim to manage disease progression and symptoms rather than provide a cure for systemic sclerosis.

Impact:

  • Provides an overview of effective treatments for SSc, including cyclophosphamide for lung involvement and specific agents for pulmonary hypertension.
  • Informs clinicians about the current evidence supporting immunosuppressive and targeted therapies in managing severe SSc complications.
  • Highlights the need for continued research into novel curative strategies for systemic sclerosis, given its poor prognosis.

Related Concept Videos

Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
iPS Cell Differentiation01:22

iPS Cell Differentiation

The ability of induced pluripotent stem cells or iPSCs to differentiate into most body cell types has stimulated repair and regenerative medicine research over the past few decades. iPSC-derived blood cells, hepatocytes, beta islet cells, cardiomyocytes, neurons, and other cell types can repair injuries or regenerate damaged tissue in diseases such as diabetes and neurodegenerative disorders.
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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...
Connective Tissue Cell Types01:22

Connective Tissue Cell Types

Connective tissue develops from the mesoderm of a developing embryo and consists of cells, fibers, and ground substance: a gel-like material containing large complexes of carbohydrates and proteins. Connective tissue was first identified as a separate tissue family in the 18th century, and Johannes Peter Muller coined the term connective tissue.
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...