Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

Abstract

Objective This study aimed to analyze the clinical characteristics and prognosis of pediatric idiopathic pulmonary hemosiderosis (IPH). Methods Pediatric IPH cases that were diagnosed at West China Second University Hospital, Sichuan University between 1996 and 2017 were reviewed. Follow-up data from 34 patients were collected. Results A total of 107 patients were included (42 boys and 65 girls). The median age was 6 years at diagnosis. The main manifestations of the patients were as follows: anemia (n = 100, 93.45%), cough (n = 68, 63.55%), hemoptysis (n = 61, 57%), fever (n = 23, 21.5%), and dyspnea (n = 23, 21.5%). There were relatively few pulmonary signs. The positive rates of hemosiderin-laden macrophages in sputum, gastric lavage fluid, and bronchoalveolar lavage fluid were 91.66%, 98.21%, and 100%, respectively. Seventy-nine patients were misdiagnosed. A total of 105 patients were initially treated with glucocorticoids, among whom 102 survived and three died. Among the followed up patients, two died and 32 survived, among whom 10 presented with recurrent episodes. Conclusions The classic triad of pediatric IPH is not always present. The rates of misdiagnosis and recurrence of IPH are high. Early recognition and adequate immunosuppressive therapy are imperative for improving prognosis of IPH.

Risk factors for recurrent pulmonary exacerbation in idiopathic pulmonary hemosiderosis

“…Our knowledge in terms of mechanisms underlying recurrent episodes in IPH is very limited 17 . The worsening of the clinical course may be due to both the primary underlying mechanism and triggering factors.…”

“…The present study indicated that recurrent episodes were frequent in children with IPH. Only one recent study has attempted to identify predictors for recurrence episodes in patients with IPH 17 . They compared results of 10 patients with recurrence episodes, and 22 patients who healed or improved.…”

“…In our study, the most common presenting features at onset were anemia, cough, dyspnea, and hemoptysis. Although hemoptysis is one of the components of the classic triad of the disease, it only occurs in around half of all patients at initial presentation 17,21 . Yet it has been reported that hemoptysis was seen in almost all adult patients during the course of the disease 3 .…”

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

“…ere is a long delay (4 months-10 years) between onset of the symptoms and diagnosis [2,5,6,10]. Especially, in young children, hemoptysis is not common as the first symptom of IPH, occurred in about 50% of patients [10,16], who swallow hemorrhage sputum. In many reports, shortness of breath, iron deficiency anemia, and alveolar infiltration on chest X-ray are typically seen [17].…”

Possible association of idiopathic pulmonary hemosiderosis with rheumatoid arthritis: A case report

“…1C and D). The patient has been closely monitored due to the high relapse incidence of IPH (18) and remained in remission at the time of the last follow-up in November 2019.…”