Diagnosis Of Idiopathic Pulmonary Hemosiderosis Research Articles

Pulmonary hemosiderosis secondary to mitral stenosis: a case report

BackgroundPulmonary hemosiderosis is a rare disease that may be idiopathic or have a secondary etiology, such as mitral stenosis. The disease is a clinical and functional consequence of iron overload in the lungs in the form of hemosiderin. The diagnosis should be considered in patients with miliary nodules on chest tomography.Case presentationWe report a case of a 30-year-old man with severe mitral stenosis who presented with chest pain and dyspnea. High-resolution chest tomography showed bilateral centrilobular micronodules. The diagnosis of pulmonary hemosiderosis secondary to mitral stenosis was suggested by transbronchial biopsy.ConclusionThe diagnosis of idiopathic pulmonary hemosiderosis requires the exclusion of other etiologies of alveolar hemorrhage, including infections and vasculitis. One possible etiology is mitral stenosis.

Idiopathic pulmonary hemosiderosis and stroke secondary to protein C deficiency in a child with Down syndrome: a case report

BackgroundPatients with Down syndrome are at a higher risk of developing autoimmune disorders such as thyroiditis, diabetes, and celiac disease compared with the general population. Although some diseases are well known to be associated with Down syndrome, others such as idiopathic pulmonary hemosiderosis and ischemic stroke due to protein C deficiency remain rare.Case presentationWe report a case of a 2.5-year-old Tunisian girl with Down syndrome and hypothyroiditis admitted with dyspnea, anemia, and hemiplegia. Chest X-ray showed diffuse alveolar infiltrates. Laboratory tests showed severe anemia with hemoglobin of 4.2 g/dl without hemolysis. A diagnosis of idiopathic pulmonary hemosiderosis was confirmed by bronchoalveolar lavage showing numerous hemosiderin-laden macrophages, with a Golde score of 285 confirming the diagnosis of pulmonary hemosiderosis. Concerning hemiplegia, computed tomography showed multiple cerebral hypodensities suggestive of cerebral stroke. The etiology of these lesions was related to protein C deficiency.ConclusionIdiopathic pulmonary hemosiderosis remains a severe disease, which is rarely associated with Down syndrome. The management of this disease in Down syndrome patients is difficult, especially when associated with an ischemic stroke secondary to protein C deficiency.

Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease without a known incidence or prevalence in adults. Our knowledge of this entity is limited as there is no prospective or retrospective study with a reasonable number of patients. The objective is to describe the demographics, clinical manifestations, diagnosis, treatment, and prognosis of adult patients with IPH. The Medline and Embase databases were searched from inception to 2021 with appropriate search formulas to identify relevant articles following strict inclusion and exclusion criteria. Statistical analyses were performed for the entire cohort and prespecified subgroups. A total of 84 patients were identified. The majority of patients were males 54/84 (64.3%). The median age was 27years. The manifesting symptoms were present in the following frequencies: anemia 76/83 (91.6%), dyspnea 71/83 (85.5%), hemoptysis 70/84 (83.3%), cough 22/84 (26.2%), and chest pain 9/84 (10.7%). The classic triad was present in 61/84 (79%) patients. The mean hemoglobin during the initial presentation was 8.4 gm/dL. A total of 16/57 (19.5%) tested positive for autoantibodies. The median delay in the diagnosis of IPH was 1.02years. Immunosuppressive therapy was prescribed in 49/79 (62%) patients, and recurrence occurred in more than half of the patients 36/66 (54.5%). A total of 63/79 (79.7%) patients were alive during the final follow-up. IPH is more common in young adults with a male predominance. A high index of suspicion is necessary to attain an early diagnosis and possibly reduce the short-term mortality of nearly 20% and long-term complications.

Puzzling (IRIDA-Like and Hemolytic) Anemia in a Child With Idiopathic Pulmonary Hemosiderosis.

Before the diagnosis of idiopathic pulmonary hemosiderosis (IPH), unexplained or puzzling anemia may precede and delay in the diagnosis of pediatric IPH is common. A 5.8 years old female child initiated with iron-refractory iron deficiency anemia-like iron deficiency and hemolytic anemia and at 6.8 years of age IPH was materialized, when the patient showed the triad signs of IPH with hemosiderin-laden alveolar macrophages in gastric aspirate. Although time to the diagnosis was previously reported to be ranged from 16 to 30 months, in our case it took 12 months from the initial anemia to IPH diagnosis.

Risk factors for recurrent pulmonary exacerbation in idiopathic pulmonary hemosiderosis.

To evaluate the risk factors of recurrent pulmonary exacerbation and poor prognosis in children with idiopathic pulmonary hemosiderosis (IPH). In this multicenter study, 54 patients with a diagnosis of IPH were included. Medical records were retrospectively reviewed from three tertiary care hospitals between 1979 and 2019. Also, current information and the long-term progress of patients was determined by contacting the families by telephone. A total of 54 children were included. The median age of onset of symptoms was 4.5 years (3 months to 15.8 years). The median time from onset to diagnosis was 0.9 years (0.25 months to 12 years). The mean number of recurrent episodes per child in the recurrence-positive group was 3.55 (1-15). Univariate analysis demonstrated that patients presenting with hypoxia or requiring transfusion at the time of presentation had significantly more recurrence episodes (p = .002). Multivariate analysis showed that the presence of hypoxia at the time of initial presentation was a significant independent predictor of recurrent episodes (p = .027). The median follow-up was 3.3 years (0.75 months to 27 years). There was a significant relationship between the presence of hypoxia, transfusion history, antinuclear antibody positivity, and elevated transaminases at the time of initial evaluation and treatment response. The present study provides substantial information regarding factors that may affect recurrent exacerbations and prognosis in children with IPH. Demonstrating hypoxia as an independent risk factor in recurrence episodes could guide physicians in the planning of treatment strategies.

IDIOPATHIC PULMONARY HEMOSIDEROSIS IN A DOWN SYNDROME PATIENT

SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Idiopathic Pulmonary Hemosiderosis (IPH) is a rare disease found primarily in children characterized by recurrent episodes of diffuse alveolar hemorrhage. The exact etiology and pathogenesis of IPH is unknown. Patients with Down Syndrome have a higher risk of developing pulmonary hemosiderosis. Additionally, in this population IPH manifests more severely. CASE PRESENTATION: Patient is 25 months old ex 36-week female with history of Trisomy 21, Atrial septal defect (ASDs) who has had a history of recurrent hemoptysis, anemia, and pulmonary infiltrates. Patient underwent extensive work-up including bronchoscopy and lung biopsy with the final diagnosis of Idiopathic Pulmonary Hemosiderosis (IPH). The patient presented to our PICU with acute alveolar hemorrhage and severe anemia secondary to RSV pneumonia and IPH recurrence despite being on maintenance therapy of prednisone and hydroxychloroquine. Patient required advanced respiratory support and was treated with a short course of pulse steroids. Patient eventually was placed on Azathioprine instead of Hydroxychloroquine with an increased Prednisone maintenance dose. DISCUSSION: IPH generally occurs in children below the age of 10 years, most commonly from 1 to 7 years. The classical triad of anemia, hemoptysis, and pulmonary infiltrates is a distinguishing characteristic of this disease; however, any one of them could be the initial manifestation. The estimated prevalence of IPH reaches 1.85 per 1,000,000 children, compared to 138.5 per 1,000,000 children with Down Syndrome. Patients with Down Syndrome tend to present earlier and more severely; they experience more dyspnea at diagnosis, more frequent secondary pulmonary hypertension (PAH), and an increased risk of fatal evolution. Systemic glucocorticoids are the mainstay of therapy for acute alveolar hemorrhage due to IPH. In a prior series of 23 children with IPH, low-dose oral prednisone was associated with prolonged survival compared with historical controls. In another series of 17 patients, long-term glucocorticoid therapy was associated with a five-year survival of 86 percent. The duration of corticosteroid treatment was variable among the patients based on clinical, radiological (alveolar infiltrates), and biological (hemoglobin, reticulocytes) evolution. Currently, there are no definitive guidelines directing use or choice of steroid-sparing medications in IPH; however, a few case reports describe the use of an immunosuppressant (cyclophosphamide, hydroxychloroquine, mycophenolate mofetil, azathioprine) in conjunction with oral glucocorticoids in the setting of respiratory failure. CONCLUSIONS: Additional studies investigating different therapeutic options are critical in order to reduce overall morbidity & mortality, particularly in children with Down Syndrome who are more predisposed to IPH relapse. Reference #1: Dr. Inderpal Randhawa Reference #2: Dr. Bugsu Ovunc Reference #3: Dr. Cyrus Shahriary DISCLOSURES: No relevant relationships by Yaron Fireizen, source=Web Response No relevant relationships by Nastasia Nianiaris, source=Web Response No relevant relationships by Inderpal Randhawa, source=Web Response No relevant relationships by Cyrus Shahriary, source=Web Response No relevant relationships by Dibanni Vasquez, source=Web Response

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

Idiopathic pulmonary hemosiderosis (IPH) is a rare interstitial lung disease, usually occurring in children or young adults. Although several studies reported on the coexistence of IPH and immune system diseases, the association between these conditions has not been well described. The present study reports on the case of a 21-year-old female patient who presented with bilateral lung abnormalities. The patient was admitted due to a 2-year history of progressive exertional dyspnea, as well as arthralgia and joint swelling in the recent 2 months. During the past 15 years, the patient had been diagnosed with anemia and received repeated blood transfusions. Serial chest CT scans indicated an interstitial pattern. On physical examination, the patient had pale skin with a hemoglobin level of 65 g/l and exhibited finger-clubbing. Arterial blood gas analysis revealed hypoxia. Anticyclic-citrullinated protein antibody and rheumatoid factor were highly positive. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. Bronchoscopy and biopsy confirmed diffuse alveolar hemorrhage. Following assessment of the etiology, the diagnosis of IPH was made by exclusion. The patient's symptoms and laboratory findings combined also confirmed the diagnosis of rheumatoid arthritis (RA). After receiving corticosteroid treatment, the patient's condition improved, and she was discharged and followed up. Based on this patient and a review of the literature, the present study demonstrated for the first time that IPH may mediate the development of an RA pathology. Therefore, early diagnosis is important for the timely management of IPH, which may also delay or even prevent the development of immune system diseases, e.g. RA, in patients with IPH. Further attention should be paid to determine the association between IPH and immune system diseases in the clinical setting.

Cryobiopsy in the diagnosis of idiopathic pulmonary hemosiderosis: a case report.

Transbronchial lung cryobiopsy (TLCB) was first described in 2009 (1) and since then it has become a common diagnostic method in interventional pulmonology. Being a safe and cost-effective method, it has been used to diagnose interstitial lung diseases (ILDs) (2,3). It is estimated that its diagnostic yield varies from 75% to 91% (4-6) and it is superior to traditional transbronchial lung biopsy (TBLB) (6,7) in terms of effectiveness. Although not yet as effective as open lung biopsy, its efficacy and safety are unquestionable (8,9). Consequently, it becomes a good alternative to open lung biopsy (10) and is always the first recommended procedure (11). While several interstitial diseases have been diagnosed by histopathological analysis of cryobiopsy samples, there are no studies, which would refer to idiopathic pulmonary hemosiderosis (IPH) confirmed by this diagnostic method. Here, we present what we believe to be the first IPH case to be confirmed by TLCB.

Tuberculosis occurring in adult idiopathic pulmonary hemosiderosis

Introduction : idiopathic pulmonary hemosiderosis (IPH) is an exceptional chronic respiratory disorder of unknown etiology. Its prevalence is estimated at 0.24 to 1.23 cases per million and nearly 80% of the cases manifest before the age of 10. Adult forms are very rare. We describe here the case of pulmonary tuberculosis occurring in adult patient with IPH. Methods : A 20-year-old woman was explored for recurrent hemoptysis, stage II dyspnea and chronic cough. Laboratory tests showed a microcytic and hypochromic hyposideremic anemia without other anomalies. X-rays and chest CT scans showed bilateral and diffuse alveolar-interstitial syndrome. Broncho alveolar lavage was suggesting an intra-alveolar hemorrhage. Systemic disease, vasculitis and severe infections were excluded and the diagnosis of IPH was confirmed by lung biopsy revealing hemosiderin-laden macrophages in the alveoli. Results : The patient received systemic glucocorticoids with a good and total response. Eight months later she was admitted because of fever, hemoptysis, marked inflammatory syndrome and leucopenia. Chest radiography and tomography were non-contributory. Bronchofibroscopy with direct exam for mycobacterium tuberculosis was positive. Under anti-tubercular therapy, the evolution was favorable. Conclusion : Lung tuberculosis associated to idiopathic pulmonary hemosiderosis is very rare. Immunodeficiency, corticotherapy as well as the underlying pulmonary lesions can support such an association.

Idiopathic Pulmonary Hemosiderosis Presenting as Anemia and Respiratory Failure in an Adult

SESSION TITLE: Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Idiopathic Pulmonary Hemosiderosis (IPH) is an extremely rare disease, which primarily occurs in children1. Its usual presentation is associated with diffuse alveolar hemorrhage (DAH)2. We describe a case of a 35-year-old patient who presented with recurrent acute hypoxemic respiratory failure requiring intubation and anemia requiring blood transfusions. Extensive workup confirmed diagnosis of DAH secondary to IPH. CASE PRESENTATION: A 35-year-old Caucasian female and active smoker presented with acute hypoxemic respiratory failure/adult respiratory distress syndrome. The patient had similar presentations requiring intubation over the course of previous two years. Her etiology was thought to be secondary to an infectious process or smoking related interstitial lung disease and was responsive to steroid therapy. Autoimmune serology was unremarkable including a vasculitis panel. The patient was also anemic requiring blood transfusions but without hemoptysis. She underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy which was consistent with DAH and hemosiderin laden macrophages. There was no evidence of vasculitis, capillaritis or infectious etiology. These findings were consistent with a diagnosis of IPH. She was started on IV Solumedrol and Imuran with excellent response to treatment. She remains stable without further respiratory compromise for the past 1 year. She is on maintenance prednisone and Imuran therapy. DISCUSSION: The etiology of IPH is unknown. It primarily presents in children and is rarely seen in adults1. When in adults, presentation is usually with hemoptysis and bilateral alveolar infiltrates. Anemia and dyspnea without hemoptysis is the common presentation in children. The etiology may be immunological causing a defect in the basement membrane of the capillary, which may explain the responsiveness to corticosteroids and immunosuppressive therapies2. Bronchoscopy with BAL showing hemosiderin-laden macrophages without any evidence of pulmonary vasculitis or capillaritis is diagnostic of the condition2. Patients respond to steroids and immunosuppressive therapies. CONCLUSIONS: IPH should be considered in any patient that presents with recurrent respiratory failure, anemia and negative vasculitis workup. Reference #1: Morgan PG, Turner-Warwick M. Pulmonary haemosiderosis and pulmonary haemorrhage. Br J Dis Chest. 1981;75(3):225. Reference #2: Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24(1):162. DISCLOSURE: The following authors have nothing to disclose: Waheeda Nazneen, Sanjay Dogra, Abdulgadir Adam, Asadulla Mohammed No Product/Research Disclosure Information

Corticosteroid in Combination with Leflunomide and Mesenchymal Stem Cells for Treatment of Pediatric Idiopathic Pulmonary Hemosiderosis.

This study evaluated the efficiency of corticosteroid, leflunomide and mesenchymal stem cells (MSCs) in the treatment of pediatric idiopathic pulmonary hemosiderosis (IPH). Ten patients were included in the study. The diagnosis of IPH was based on clinical symptoms, laboratory examinations and pulmonary hemosiderosis. Induction therapy consisted of methylprednisolone pulse therapy, followed by prednisone plus leflunomide. Maintenance therapy consisted of low-dose prednisone, leflunomide and administration of MSCs. All the patients achieved complete response after treatment with corticosteroid, leflunomide and MSCs. The median follow-up was 23 months (range: 4-34 months). Moreover, administration of MSCs induced an increase in the percentage of CD4+ CD25+ regulatory T cells but a decrease in the percentage of Th17 cells. Treatment with corticosteroid, leflunomide and MSCs for pediatric IPH was safe and effective.

Neonatal pulmonary hemosiderosis.

Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage). The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images) is strongly suggestive.

Idiopathic Pulmonary Hemosiderosis in Adults: A Case Report and Review of the Literature

Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon exclusion of all other possible causes of DAH in combination with specific pathologic findings revealing bland alveolar haemorrhage with absence of vasculitis and/or accumulation of immune complexes within lung parenchyma. Here we describe a rare case of idiopathic pulmonary hemosiderosis in an otherwise healthy 27-year-old Greek male patient with relapsing episodes of fever accompanied by general fatigue and discomfort. He was at this time point a light smoker and had been hospitalised once in the past for similar symptoms. His iron deficiency anemia coupled with chest high-resolution computed tomography and bronchoalveolar lavage revealed findings compatible with diffuse alveolar hemorrhage. After excluding all other sources of bleeding through extensive gastrointestinal workup and thorough immunologic profile, video-assisted thoracic lung biopsy was performed and the diagnosis of Idiopathic Pulmonary Hemosiderosis was established. Patient was treated with high doses of oral corticosteroids, leading to clinical response. We highlight the need for vigilance by the respiratory physician for the presence of DAH, a challenging, acute condition requiring early recognition along with identification of the underlying syndrome and appropriate treatment to achieve optimal results.

Idiopathic pulmonary hemosiderosis in adult

Idiopathic pulmonary hemosiderosis (IPH) is a rare unknown origin disease characterized by diffuse alveolar hemorrhage (DPH). It rarely occurs in adults. We report a case of 50-year-old man, hospitalized with acute respiratory insuffiency. The chest radiograph and computed tomography scan showed bilateral interstitial and ground-glass opacities. The diagnosis of IPH was confirmed by broncho-alveolar lavage (BAL) and surgical pulmonary biopsy. There was no sign of capillaritis or granuloma in the anatomopathologic study. No remission was obtained after two months of high-dose corticosteroid therapy

Maintenance therapy with dose‐adjusted 6‐mercaptopurine in idiopathic pulmonary hemosiderosis

There are challenges for diagnosis and treatment of idiopathic pulmonary hemosiderosis (IPH). This clinical trial was to review the diagnosis and evaluate the efficacy of maintenance therapy with dose-adjusted 6-mercaptopurine (6MP) in IPH children. Fifteen children were enrolled. Prednisone was administered at 2 mg/kg/day for 4 weeks in acute phase of the disease followed by taper. 6MP was also started at 60 mg/m(2)/day simultaneously and continued for 3 years in outpatient. The delay in diagnosis of IPH is common and probably due to a lack of classical triad of IPH in most children. All the patients exhibited response to the initial treatment. Only one of eight patients with relative leukopenia on 6MP maintenance recurred while 5 of 7 others recurred (P < 0.05) during median 4.5-year follow-up. Of the latter five patients who recurred, 4 remained recurrence-free after adjusting the dose of 6MP upwards to keep relative leucopenia. It suggests that children with IPH could achieve steroid-free long term remission on 6MP maintenance therapy, and relative leukopenia on 6MP might be a simple maker of predicting clinical response in most IPH children.

Co-occurrence pulmonary haemosiderosis with coeliac disease in child

Idiopathic pulmonary haemosiderosis (IPH) is a rare and serious disorder in children of unknown aetiolopathogeny. Association of IPH and coeliac disease (CD) is even rarer. Immunological origin of IPH is now well accepted. We report the case of an 11-year-old female admitted for evaluation of recurrent streaky haemoptysis that had been evolving over the previous 9 months. Physical examination revealed weight loss with normal weight, but there was cutaneous and mucosal pallor due to severe anaemia (haemoglobin 4.6g/dl). The chest X-rays showed unilateral alveolo-intertitial infiltrate. Broncho-alveolar lavage revealed 70% haemosiderin-laden macrophages. The diagnosis of IPH was made. Since severe anaemia is disproportionate to radiologic findings, searching associated CD was performed and then confirmed by biological and histological examinations. A gluten-free diet was initiated. Evolution was favourable. Looking for especially CD in IPH should be systematic, even in the absence of gastrointestinal symptoms.

IDIOPATHIC PULMONARY HEMOSIDEROSIS IN CHILDREN: A ROMANIAN EXPERIENCE

INTRODUCTION: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease with unknown cause and variable outcome. It is characterized by recurrent episodes of severe hypochromic anemia, alveolar bleeding, and typical radiologic findings. OBJECTIVE: The objective of this study was to develop an early diagnosis of IPH with real therapeutic benefits. METHODS: We conducted a multicenter, retrospective, and prospective study using patients who were admitted to 3 Romanian pediatric clinics between 1984 and 2006. Secondary causes of pulmonary hemosiderosis were excluded. RESULTS: Fifteen patients received a diagnosis of IPH during a 22-year period (1984–2006). The symptoms started at a mean age of 6.8 years (range: 9 months to 13 years), with a mean delay of 2.4 years before diagnosis. From the beginning, all patients had anemia, and only 6 children presented with pulmonary symptoms as well. The classical triad (anemia, hemoptysis, and pulmonary infiltrates) was found from early in the disease in only 4 patients. The majority of patients' disease was diagnosed by bronchoalveolar lavage, and 3 were diagnosed at necropsy. Eight patients died in a period of 1 to 3 years from the diagnosis. The clinical course was variable: treatment with corticosteroids alone was not effective because 12 patients continued to have recurrent bleeding. Three patients who received immunosuppressive agents had a better outcome. CONCLUSIONS: IPH is a severe condition with variable prognosis and has a better outcome when diagnosis is made at an early age. We believe that it is necessary to include in the screening of any severe, recurrent, hypochromic anemia a well-interpreted chest radiograph and to look for hemosiderin-laden phages in bronchoalveolar lavage.

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

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.

Coeliac disease as a cause of unusually severe anaemia in a young man with idiopathic pulmonary haemosiderosis

Idiopathic pulmonary haemosiderosis (IPH) is characterized by a triad of recurrent episodes of alveolar haemorrhage, haemoptysis and iron deficiency anaemia. The combination of IPH and coeliac disease (CD) is extremely rare though both diseases may have a common pathogenetic link. As illustrated by our case CD should be specifically looked for in patients with IPH, especially those in whom the severity of anaemia is disproportionate to radiologic findings even in the absence of gastrointestinal symptoms since both diseases may benefit from a gluten-free diet.

Hemosiderosis pulmonar idiopática: Evolución de 5 niños

Idiopathic pulmonary haemosiderosis (IPH) is a rare and serious disorder characterised by diffuse alveolar haemorrhage. Its aetiology is unknown but owing to its unpredictable prognosis long term treatment is justified. Objective: to describe the clinical and therapeutic characteristics of children with IPH during 5 years of follow up. Design: this prospective study reports our experience with 5 children (3 males) aged 9 to 21 years with IPH. In 4 the initial diagnosis was pneumonia, with an average delay in the diagnosis of IPH of 2.5 years. 3 children came from agricultural zones where pesticides are frequently used. All children were treated with a standard protocol of prednisone 2 mg/kg/day for 1 month followed by 1 mg/kg every other day, plus inhaled budesonide 800-2 400 ug/day and the addition of azathioprine 2 mg/kg/day or chloroquine 10 mg/kg/day. Secondary effects included osteoporosis in 5 children, Cushing syndrome in 3 and retrocapsular opacities in 2. After 5 years of treatment 2 children stopped having bleeding episodes, 3 had in general mild episodes in whom 2 had serious bleeding events, one needing mechanical ventilation. There were no deaths. One patient became pregnant without ill effects and delivered a healthy baby. Initial pulmonary function (PFT) tests were normal in 3, mildly restrictive in 1 and mildly obstructive in 1, however during the follow-up period there were changes in the PFT in 2 patients, from normal to mildly restrictive in 1 and from mildly to moderately obstructive in 1. Intial pulmonary CT were normal in 2, 2 had a pattern of alveolar infiltrates and one interstitial infiltrates, follow up CT scans remained normal in 2 patients and showed interstitial infiltrates in the rest. Conclusions: Using this protocol in a small series of IPH there were no deaths after five years of follow up. We speculate that the use of inhaled steroids can reduce the secondary effects of oral steroids in IPH.