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Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature

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Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease without a known incidence or prevalence in adults. Our knowledge of this entity is limited as there is no prospective or retrospective study with a reasonable number of patients. The objective is to describe the demographics, clinical manifestations, diagnosis, treatment, and prognosis of adult patients with IPH. The Medline and Embase databases were searched from inception to 2021 with appropriate search formulas to identify relevant articles following strict inclusion and exclusion criteria. Statistical analyses were performed for the entire cohort and prespecified subgroups. A total of 84 patients were identified. The majority of patients were males 54/84 (64.3%). The median age was 27 years. The manifesting symptoms were present in the following frequencies: anemia 76/83 (91.6%), dyspnea 71/83 (85.5%), hemoptysis 70/84 (83.3%), cough 22/84 (26.2%), and chest pain 9/84 (10.7%). The classic triad was present in 61/84 (79%) patients. The mean hemoglobin during the initial presentation was 8.4 gm/dL. A total of 16/57 (19.5%) tested positive for autoantibodies. The median delay in the diagnosis of IPH was 1.02 years. Immunosuppressive therapy was prescribed in 49/79 (62%) patients, and recurrence occurred in more than half of the patients 36/66 (54.5%). A total of 63/79 (79.7%) patients were alive during the final follow-up. IPH is more common in young adults with a male predominance. A high index of suspicion is necessary to attain an early diagnosis and possibly reduce the short-term mortality of nearly 20% and long-term complications.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

Abbreviations

AAV:

ANCA-associated vasculitis

AM:

Alveolar macrophage

ANCA:

Antineutrophil cytoplasmic antibody

AZA:

Azathioprine

BAL:

Bronchoalveolar lavage

CD:

Celiac disease

CS:

Corticosteroid

DAH:

Diffuse alveolar hemorrhage

GFD:

Gluten-free diet

HLM:

Hemosiderin-laden macrophages

IPH:

Idiopathic pulmonary hemosiderosis

SLB:

Surgical lung biopsy

TBLB:

Transbronchial lung biopsy

VATS:

Video-assisted thoracoscopic surgery

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Authors and Affiliations

  1. Department of Pulmonary and Critical Care Medicine, Ozarks Medical Center, 1100 Kentucky Avenue, West Plains, MO, 65775, USA

    Biplab K. Saha

  2. Department of Critical Care Nursing, Goldfarb School of Nursing, Barnes Jewish College, St. Louis, MO, USA

    Alyssa Bonnier

  3. Department of Medicine, Saha Clinic, Narail, Bangladesh

    Santu Saha & Baidya Nath Saha

  4. Department of Pulmonary and Critical Care Medicine, Albany Medical Center, Albany, NY, USA

    Boris Shkolnik

Authors
  1. Biplab K. Saha
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  2. Alyssa Bonnier
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  3. Santu Saha
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  4. Baidya Nath Saha
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  5. Boris Shkolnik
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Contributions

BKS, AB, and SS planned the study. BKS, AB, and SS collected the data. BKS, AB, SS, and BS performed data synthesis. BKS, AB, SS, BS, and BNS prepared the initial manuscript. All the authors contributed to the finalization of the manuscript.

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Correspondence to Biplab K. Saha.

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Saha, B.K., Bonnier, A., Saha, S. et al. Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature. Clin Rheumatol 41, 1627–1640 (2022). https://doi.org/10.1007/s10067-022-06104-3

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  • DOI: https://doi.org/10.1007/s10067-022-06104-3

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