Systemic sclerosis

  title={Systemic sclerosis},
  author={Christopher P. Denton and Dinesh Khanna},
  journal={The Lancet},

Systemic Sclerosis and Pulmonary Disease.

  • K. Ngo
  • Medicine
    Advances in experimental medicine and biology
  • 2021
There are inherent challenges in the management for both pulmonary hypertension and interstitial lung disease but with the focus on early diagnosis for each of these lung complications, treatment may have a higher chance of efficacy.

Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review

Current treatment options for scleroderma are discussed, encompassing old, new, and future potential treatment options, as well as data from new clinical trials that have the potential to modify the disease process and improve long-term outcomes in SSc.

Does early systemic sclerosis really exist?

Therapeutic Approaches to Systemic Sclerosis: Recent Approvals and Future Candidate Therapies

Two targeted therapies, tocilizumab and nintedanib, have recently been approved by the American Food & Drug Administration to limit the decline of lung function in patients with SSc-associated interstitial lung disease, demonstrating that better understanding of the disease pathogenesis with the identification of key targets can lead to therapeutic advances in the management of some visceral manifestations of the Disease.

Lung involvement in systemic sclerosis

Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review

Cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, and dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.

Antifibrotics in systemic sclerosis.

Tocilizumab Treatment of Patients with Systemic Sclerosis: Clinical Data

The interleukin-6 receptor-alpha antagonist tocilizumab has demonstrated promising efficacy in a phase 2 trial and is being investigated in a phases 3 trial, and tocilIZumab for the treatment of SSc in clinical trials is discussed.



Kidney involvement in systemic sclerosis: From pathogenesis to treatment

Except for angiotensin-converting enzyme inhibitors in scleroderma renal crisis, no other treatment is currently recommended for treatment of kidney involvement in systemic sclerosis, and further studies are necessary to investigate its prognostic impact.

Gastrointestinal Manifestations of Systemic Sclerosis

The current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways are presented.

[Pathogenesis of systemic sclerosis].

  • M. FabriT. Krieg
  • Medicine, Biology
    Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
  • 2007
Multiple mediators with fibrotic effects such as Platelet Derived Growth Factor, Transforming Growth Factor-beta and Connective Tissue Growth Factor have been characterized and have become targets for innovative therapeutic strategies that might lead to specific treatments for systemic sclerosis.

Ongoing clinical trials and treatment options for patients with systemic sclerosis–associated interstitial lung disease

Newer agents targeting the pathogenic mechanisms underlying SSc-ILD, including fibrotic and inflammatory pathways, lymphocytes, cell–cell and cell–extracellular membrane interactions, hold promise for better treatment outcomes, including improved lung function, patient-related outcomes and quality of life.

Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease.

The adverse-event profile of nintedanib observed in this trial was similar to that observed in patients with idiopathic pulmonary fibrosis; gastrointestinal adverse events, including diarrhea, were more common with nintinganib than with placebo.

Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group

Data favour distinguished decision-making, pointing to either watchful waiting and close monitoring in the early stages or start of immunosuppressive treatment in moderately impaired lung function, and do not support liberal use of GC in SSc-ILD.

Autologous Hematopoietic Stem Cell Transplantation for Treatment of Systemic Sclerosis

Current evidence suggests that SSc patients who are most likely to benefit from AHSCT are early, active, with rapidly progressing diffuse skin disease, and mild involvement of internal organs.

Controversies on the Use of Steroids in Systemic Sclerosis

The arguments for and against corticosteroids in this clinical situation are presented, along with two case histories to illustrate the clinical dilemma.

Cardiac complications of systemic sclerosis.

Treatment of early abnormalities with calcium channel blockers and angiotensin-converting enzyme inhibitors may improve myocardial perfusion and function, while standard management of overt cardiac disease is equally appropriate in the SSc population.