Long‐term prednisone and azathioprine treatment of a patient with idiopathic pulmonary hemosiderosis

Rossi, Giovanni; Balzano, Emma; Battistini, Elena; Oddera, Susanna; Marchese, Patrizia; Acquila, Maura; Fregonese, B; Mori, Pier Giorgio

doi:10.1002/ppul.1950130310

Cited by 39 publications

(10 citation statements)

References 16 publications

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“…Other immunosuppressive agents such as azathioprine, cyclophosphamide, and hydroxychloroquine have also been used alone or in combination with oral corticosteroids. [7][8][9][10][11] Low-dose oral corticosteroids, azathioprine, or methotrexate are used in maintenance phase. As there is lack of large patient series and inadequate follow-up in previous studies, the prognosis of IPH remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Siu

Lam

2015

Hong Kong Med J

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Section: Discussionmentioning

confidence: 99%

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Siu

Lam

2015

Hong Kong Med J

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“…Inhaled steroids also have been tried, but insufficient experience exists to date [91,92]. Other immunosuppressant agents, including azathioprine, hydroxychloroquine, cyclophosphamide, and methotrexate, have been tried with variable results [11,22,[93][94][95][96][97][98][99][100][101][102]. Among them, azathioprine in combination corticosteroids might be the best therapeutic regimen, especially in preventing IPH exacerbations.…”

Section: Treatmentmentioning

confidence: 99%

Idiopathic pulmonary haemosiderosis revisited

Ioachimescu¹,

Sieber²,

Kotch³

2004

European Respiratory Journal

200

199

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Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli.Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderinladen alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/ granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.

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“…There are no controlled studies or large series of patients with IPH, so therapy is based on observational studies on relatively small numbers of patients. Treatment focuses mainly on immunosuppressant therapy, such as systemic glucocorticoids, 7 hydroxychloroquine, 8 cyclophosphamide, 9 and azathioprine, 10 with variable results. Lung transplantation has also been reported, but was accompanied by recurrence of IPH.…”

Section: Discussionmentioning

confidence: 99%

Extracorporeal membrane oxygenation to rescue profound pulmonary hemorrhage due to idiopathic pulmonary hemosiderosis in a child

Sun¹,

Tseng²,

Huang³

et al. 2006

Pediatric Pulmonology

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Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) in pediatric patients. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure with associated multiple organ failure often occurs. We report the case of an 11-year-old girl who developed severe hypoxemic respiratory failure and pulmonary hemorrhage. Extracorporeal membrane oxygenation (ECMO) was instituted on the next day because medical treatment and mechanical ventilation failed to maintain oxygenation. She was successfully decannulated 5 days later without ECMO-related complications. Four months later, she was intubated again and the diagnosis of IPH was made by open lung biopsy. She was treated with systemic steroid therapy and discharged smoothly. We suggest that ECMO provides a chance of recovery and survival for patients with IPH, even if accompanied by severe pulmonary hemorrhage.

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Long‐term prednisone and azathioprine treatment of a patient with idiopathic pulmonary hemosiderosis

Cited by 39 publications

References 16 publications

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Idiopathic pulmonary haemosiderosis revisited

Extracorporeal membrane oxygenation to rescue profound pulmonary hemorrhage due to idiopathic pulmonary hemosiderosis in a child

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