Iron therapy as a novel treatment of scleroderma-related pulmonary hypertension: A case report and literature review Journal Article


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
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  1. Kevin Gerard Lazo
    8 Lazo
  2. Karen A Wong
    9 Wong