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This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical\u00a0and radiological characteristics, treatment, and prognosis. Additionally, the association between Celiac Disease (CD) and IPH is carefully evaluated. IPH is a frequently misdiagnosed disease. The delay in the diagnosis of IPH is often significant but fortunately, appears to have decreased in recent years. IPH in adults and children have distinct demographic preferences. The autoantibodies are common in IPH but with a definite difference between the adult and pediatric populations. The definitive diagnosis of IPH requires lung biopsy and careful exclusion of all competing diagnoses, even with lung biopsy showing bland pulmonary hemorrhage. The presence of nonspecific inflammatory cells or lymphoid aggregates may suggest a secondary immunologic phenomenon and needs careful evaluation and follow\u2010up. A substantial number of patients suffer from coexisting CD, also known as Lane\u2212Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet\u00a0can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids\u00a0are the backbone of IPH therapy. Recently utilized experimental treatment options include mesenchymal stem cell transplant, liposteroid and bronchial artery embolization. The immunosuppression should be adjusted to achieve optimal disease control. Patients may progress to end\u2010stage lung disease despite all measures, and lung transplantation may be the only viable option.<\/jats:p>","DOI":"10.1002\/ppul.26230","type":"journal-article","created":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T04:28:45Z","timestamp":1667449725000},"page":"382-391","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3595-8804","authenticated-orcid":false,"given":"Biplab K.","family":"Saha","sequence":"first","affiliation":[{"name":"Department of Pulmonary, Critical Care and Sleep Medicine University of Florida  Gainesville Florida 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