What is the role of serologic analyses for idiopathic pulmonary arterial hypertension (IPAH)?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Excluding autoimmune disorders is an important part of the workup in a patient with suspected pulmonary hypertension. Reportedly, up to 40% of patients with IPAH have a positive finding on an antinuclear antibody (ANA) assay but no other clinical manifestations of autoimmune disease.

Most connective-tissue diseases associated with pulmonary artery hypertension are diagnosed on the basis of clinical findings (ie, physical examination), with serology results used as adjunctive confirmation of the disease. These serologies may include rheumatoid factor (RF), anti-neutrophil cytoplasmic antibody (ANCA), and anti-topoisomerase antibody (SCL70), (Also see Scleroderma.)


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