Long-Term Outcome of Idiopathic Pulmonary Hemosiderosis in Children

Abstract

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.

Red blood cells increase secretion of matrix metalloproteinases from human lung fibroblasts in vitro

“…Interestingly, a role for both MMPs and neutrophils has also been suggested in these conditions (10,15,41,45,49). Patients with idiopathic pulmonary hemosiderosis frequently develop fibrosis in the lung parenchyma (7,31), suggesting a possible role of RBC in ECM remodeling. Although the mechanisms that regulate the pathological ECM turnover in different lung diseases are incompletely understood, it is likely that MMPs play an important role.…”

“…The interplay between inflammatory and mesenchymal cells in the repairing or remodeling process is crucially important in lung disorders. In IPF and asthma, diseases associated with inflammation and fibrosis, imbalance between MMPs and TIMPs can result in fibrosis (28,30,39,42), and patients with idiopathic pulmonary hemosiderosis frequently develop pulmonary fibrosis (7,31). In contrast, patients with chronic obstructive pulmonary disease and emphysema, diseases characterized by inflammation and increased matrix degradation, also have altered synthesis of MMPs and decreased TIMP synthesis (4,26).…”

Idiopathic pulmonary haemosiderosis revisited

“…Some believed in an autoimmune aetiopathogenesis based on the demonstration of plasma circulating immune complexes [29], though the immunohistochemical examination of lung tissue generally has not supported an immunological pathogenesis [23,24,[27][28][29]. Interestingly, about a quarter of the patients who survive w10 yrs with this disease subsequently develop some form of autoimmune disease [30][31][32]. The accumulation of neutrophils in the alveoli also may play a role [33].…”

Hidroxicloroquina na hemossiderose pulmonar idiopática – Caso clínico

“…Existem dados limitados sobre a epidemiologia desta entidade, dado ser uma doença rara. A maioria das publicações descreve pequenas séries [4][5][6][7][8][9] ou casos limitados [10][11][12][13][14][15] . Em 80% dos casos ocorre na população pediátrica 12 .…”

“…Muitos autores sugerem mecanismos imunológicos na patogénese da HPI 2,6,7,12,15 . A determinação dos anticorpos citoplasmáticos anti-neutrófilos citoplasmáticos (C-ANCA) tem sido recomendado a todos os doentes com síndro-mas alveolares pulmonares hemorrágicos, servindo como um sinal de mau prognós-tico [9][10][11][12][13][14][15] . Os tratamentos disponíveis, escassos e não específicos, e a história natural da HPI, caracterizada por exacerbações frequentes e remissões, torna difícil avaliar a terapêutica instituída [2][3][4]16 .…”