Aggressive corticosteroid treatment in childhood idiopathic pulmonary hemosiderosis with better outcome.

Idiopathic pulmonary hemosiderosis (IPH) is a rare but fatal disease characterized by a triad of anemia, hemoptysis, and increased pulmonary infiltration. This study is aimed to review the clinical manifestations, diagnostic tools, medication and outcome of childhood IPH in Taiwan. We retrospectively enrolled the patients less than 18 years old in National Taiwan University Hospital in the past 30 years. The clinical data were collected and analyzed. All of the twelve children diagnosed with IPH had anemia and increased pulmonary infiltration, eight had hemoptysis, and ten were confirmed with detection of hemosiderin-laden macrophages. The mean age at diagnosis were 4.9 (interquartile range 2.5-6.3) years old. Patients with high dose corticosteroid (CS, ≥ 1 mg/kg/day prednisolone equivalent) treatment had lower odds ratio for ICU admission and significant higher Hb recovery rate than those with mild disease activity not receiving high dose CS treatment (p = 0.011). The only factor that is significantly associated with persistent anemia is the usage of high dose CS (p < 0.001) after adjusting for hemoptysis, fulfilling triad, serum ferritin level, and ICU admission by multiple regression. The only factor that is significantly associated with ICU admission is the presence of microorganism yielded in sputum (p < 0.001) after adjusting for fever, serum ferritin level, usage of invasive MV, and high dose CS treatment days. The aggressive high dose CS therapy might prevent ICU admission and improve anemia. Aggressive high dose CS treatment is suggested in IPH patients regardless of the disease activity.

Yang CT ，Chiang BL ，Wang LC 《JOURNAL OF THE FORMOSAN MEDICAL ASSOCIATION》

Anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases.

Chen KC ，Hsiao CC ，Huang SC ，Ko SF ，Niu CK ... -  《-》

Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children.

Kabra SK ，Bhargava S ，Lodha R ，Satyavani A ，Walia M ... -  《INDIAN PEDIATRICS》

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients.

Zhang Y ，Luo F ，Wang N ，Song Y ，Tao Y ... -  《-》

Idiopathic pulmonary hemosiderosis: A state of the art review.

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH). Patients with IPH usually present with hemoptysis, and the diagnosis is often delayed by years. Patients often present with intermittent episodes of hemoptysis interspersed between periods of relative normalcy. However, massive hemorrhage resulting in acute respiratory failure and non-remitting hemoptysis have also been described. The classic triad includes hemoptysis, radiologic lung infiltrate, and iron deficiency anemia. Several hypotheses regarding the pathogenesis of IPH have been proposed. These risk factors include an autoimmune, allergic or genetic predisposition, and possible environmental exposure. Since IPH appears to be responsive to corticosteroids, the autoimmune hypothesis is considered to play a crucial role. A diagnosis of IPH requires exclusion of other etiologies of DAH, including infection, medications, toxic inhalation, vasculitis, and anti-glomerular basement membrane disease, among others. Histologically, IPH is characterized by the presence of hemosiderin-laden macrophages in the alveolar space without any evidence of vasculitis or immunocomplex deposition. Corticosteroid therapy represents the primary modality of treatment. Other immunosuppressive medications have also been used with varying success, especially in the setting of steroid-refractory disease. The prognosis of IPH in adults is somewhat better compared to the pediatric population. The severity of the initial presentation does not predict future outcomes. Which risk factors and patient characteristics are associated with a poor outcome are also unknown. More research is necessary to elucidate the pathophysiology and appropriate treatment.

Saha BK 《-》