Long-term liposteroid therapy for idiopathic pulmonary hemosiderosis

Doi, Takehiko; Ohga, Shouichi; Ishimura, Masataka; Takada, Hidetoshi; Ishii, Kanako; Ihara, Kenji; Nagai, Hideyuki; Hara, Toshiro

doi:10.1007/s00431-013-2065-9

Cited by 22 publications

(31 citation statements)

References 29 publications

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“…Immunosuppressive agents are the second choice of drugs, especially in steroid-dependence or steroid-resistance cases [5,7,10,15,19]. However, immunosuppressants cause the change of immune system, increase risk of infection that may trigger IPH and the possible risk of developing malignancy [8,12,20]. As an another option of treatment, Ohga et al proposed the liposteroid therapy for the improved outcome of patients with refractory IPH [8,21].…”

Section: Discussionmentioning

confidence: 99%

“…Dexamethasone has higher affinity for the corticosteroid receptor than prednisolone or methylprednisolone and has stronger anti-inflammatory effects [8]. Liposteroid is dexamethasone palmitate, which is a lipid emulsion containing dexamethasone.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, liposteroid has lipo-based, palmitate, which is more harmful to non-adipose tissue, is easily taken up by macrophages, and induces strongly apoptosis of macrophages [24,25]. Doi et al suggested that low-dose liposteroid therapy accumulates effectively in the hemorrhagic inflamed sites of the lung, reduces the chance of high-dose corticosteroid therapy and prevents adverse effects [8]. Liposteroid was an agent to prevent acute and chronic bleeding and also contributed to wean steroids in our case.…”

Section: Discussionmentioning

confidence: 99%

“…Other diagnostic methods can be conducted for confirmation of hemosiderin-laden macrophages (siderophages) by bronchoalveolar lavage, sputum, or gastric lavage analysis [5][6][7]. Systemic corticosteroid is the first line treatment of IPH for acute bleeding [2,6,8,9]. Immunosuppressants, including azathioprine, hydroxychloroquine, and cyclophosphamide have been proposed in patients with unfavorable response to corticosteroid [1,4,10,11].…”

Section: Introductionmentioning

confidence: 99%

“…Various therapeutic trials have attempted to improve the prognosis with IPH. Nevertheless, no effective maintenance therapy has been established for children with refractory IPH [7,8]. Liposteroid, dexamethasone palmitate, has been introduced as a new effective therapy [8].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome

Tobai

Yano

Sato

et al. 2020

Case Reports in Pediatrics

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Idiopathic pulmonary hemosiderosis (IPH) is a rare and life-threatening disorder. Early diagnosis and appropriate management are essential for their better prognosis and patients’ quality of life (QOL). It is considered that Down syndrome patients with IPH have a worse prognosis compared to other IPH cases. A 2-year-old girl with Down syndrome received the diagnosis of IPH after two episodes of massive pulmonary hemorrhage requiring assist ventilation, who suffered from recurrent IPH during tapering period of oral corticosteroid, started liposteroid therapy. We report here a case of successful control of recurrent IPH and improved QOL enormously with tapering dose of corticosteroid after starting liposteroid therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome

Tobai

Yano

Sato

et al. 2020

Case Reports in Pediatrics

Self Cite

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Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

Saha

Aiman

Chong

et al. 2022

Pediatric Pulmonology

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This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhage in children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical and 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-up. A substantial number of patients suffer from coexisting CD, also known as Lane−Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids are 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 endstage lung disease despite all measures, and lung transplantation may be the only viable option.

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Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions

Saha

Milman

2020

Clin Rheumatol

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Long-term liposteroid therapy for idiopathic pulmonary hemosiderosis

Abstract: The liposteroid therapy might improve the survival of IPH patients with reducing the adverse effects of steroids, although prospective control studies are needed.

Cited by 22 publications

References 29 publications

Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome

Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions

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