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Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database.

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Renal involvement in systemic sclerosis.

Marc Scheen1, Arnaud Dominati2, Valérie Olivier1

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Summary
This summary is machine-generated.

Systemic sclerosis can cause various kidney problems, including scleroderma renal crisis (SRC). Early diagnosis and treatment of these renal manifestations in systemic sclerosis are crucial for better patient outcomes.

Keywords:
ACEIFibrinoid necrosisRenal pathologyRenal vasculopathyScleroderma renal crisisSystemic sclerosis

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Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • Systemic sclerosis is a rare autoimmune disease causing fibrosis.
  • Renal involvement is common and associated with poor prognosis.
  • Scleroderma renal crisis (SRC) is a major renal complication, though ACE inhibitors have improved outcomes.

Purpose of the Study:

  • To review the main renal manifestations in systemic sclerosis.
  • To discuss recent epidemiological and pathophysiological data.
  • To highlight diagnostic challenges for clinicians.

Main Methods:

  • Literature review of systemic sclerosis and renal disease.
  • Analysis of epidemiological and pathophysiological data.
  • Discussion of clinical diagnostic challenges.

Main Results:

  • Renal manifestations range from decreased GFR and proteinuria in SRC to rarer conditions like antiphospholipid antibody nephropathy and ANCA-associated vasculitis.
  • Pathophysiology involves renal vasculopathy, complement system, endothelin-1, autoimmunity, and pro-inflammatory cytokines.
  • Despite improved SRC outcomes, a wide differential diagnosis for renal disease in systemic sclerosis remains challenging.

Conclusions:

  • Systemic sclerosis presents diverse renal manifestations requiring careful diagnosis.
  • Understanding the complex interplay of factors is key to managing renal disease in systemic sclerosis.
  • Further research is needed to address diagnostic and therapeutic challenges.