Severe pulmonary hypertension as the initial manifestation of systemic lupus erythematosus: a case report and review of the literature

Clin Exp Rheumatol. 2014 Mar-Apr;32(2):267-74. Epub 2013 Dec 16.


Severe pulmonary arterial hypertension (PAH) is rarely observed as the initial manifestation of systemic lupus erythematosus (SLE), and the diagnosis is often delayed. Here we present the case of a 32-year-old woman with severe PAH as the initial manifestation of SLE, who was successfully treated with mycophenolate mofetil and cyclosporine. This case offered the opportunity to critically review the epidemiology data, predictive markers, and pathogenic pathways of SLE-associated PAH (SLE-PAH) in relation to the currently available therapeutic options and to the main clinical trials of the last 10 years focused on the treatment of SLE-PAH. Mycophenolate mofetil and cyclosporine - currently used in the maintenance phase of the disease in certain clinical settings - should be considered, as an alternative to cyclophosphamide, in future clinical trials aimed at evaluating the most effective treatment of SLE-PAH at presentation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antibodies, Antiphospholipid / blood*
  • Autoimmunity / drug effects
  • Biomarkers / blood
  • Clinical Trials as Topic
  • Cyclosporine / administration & dosage*
  • Delayed Diagnosis / prevention & control
  • Disease Management
  • Endothelin-1 / blood*
  • Familial Primary Pulmonary Hypertension
  • Female
  • Humans
  • Hypertension, Pulmonary* / drug therapy
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / immunology
  • Hypertension, Pulmonary* / physiopathology
  • Immunosuppressive Agents / administration & dosage
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / drug therapy
  • Lupus Erythematosus, Systemic* / immunology
  • Lupus Erythematosus, Systemic* / physiopathology
  • Mycophenolic Acid / administration & dosage
  • Mycophenolic Acid / analogs & derivatives*
  • Predictive Value of Tests
  • Rheumatoid Factor / blood*
  • Severity of Illness Index
  • Treatment Outcome


  • Antibodies, Antiphospholipid
  • Biomarkers
  • Endothelin-1
  • Immunosuppressive Agents
  • Cyclosporine
  • Rheumatoid Factor
  • Mycophenolic Acid