Authors: | Neumann, M.; Wong, K. A.; Lazo, K.; Stover, D. |
Article Title: | Iron therapy as a novel treatment of scleroderma-related pulmonary hypertension: A case report and literature review |
Abstract: | Pulmonary arterial hypertension (PAH) is the leading cause of death in patients with systemic sclerosis (SSc), with a 3-year mortality of 40%–50% despite optimal therapy. Treatment mirrors that of idiopathic PAH and is often ineffective. This is a case report of a patient with SSc evaluated for progressive dyspnoea with exertion and found to have elevated pulmonary artery systolic pressures (PASPs). She received ferritin-targeted iron infusions as a novel treatment of suspected SSc-associated PAH, with subsequent resolution of respiratory symptoms and PASPs that normalized. We review PAH especially associated with SSc, its treatment and identify a possible novel therapeutic approach for those with PAH-SSc. © 2022 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology |
Keywords: | clinical article; aged; case report; hemoglobin; dyspnea; colonoscopy; echocardiography; gastroesophageal reflux; lung function test; immunosuppressive agent; ferritin; raynaud phenomenon; pulmonary hypertension; histamine h2 receptor antagonist; lung diffusion capacity; esophagogastroduodenoscopy; scleroderma; iron therapy; systemic sclerosis; human; female; article; ferrous gluconate; iron saturation; pulmonary artery systolic pressure; syndrome crst; transferrin saturation |
Journal Title: | Respirology Case Reports |
Volume: | 10 |
Issue: | 2 |
ISSN: | 2051-3380 |
Publisher: | John Wiley & Sons Ltd. |
Date Published: | 2022-02-01 |
Start Page: | e0904 |
Language: | English |
DOI: | 10.1002/rcr2.904 |
PROVIDER: | scopus |
PMCID: | PMC8767940 |
PUBMED: | 35079404 |
DOI/URL: | |
Notes: | Article -- Export Date: 1 March 2022 -- Source: Scopus |