Idiopathic Pulmonary Hemosiderosis Research Articles

Pulmonary hemosiderosis in a child

Idiopathic pulmonary hemosiderosis is a rare respiratory disease that often beginning before the age of 10 years. In the pediatric population, it can be manifested only by isolated iron deficiency anemia, without association of other symptoms, sometimes with late diagnosis. We will present a clinical case of pulmonary hemosiderosis with metabolic damage, characterized by the accumulation of hemosiderin in alveolar macrophages and increased levels of ferritin. The progression of lung dysfunction is evaluated imaging by lung CT.

Clinical features, paraclinical characteristics, and etiology of mediastinal bleeding diffusion in children at the Vietnam National Children’s Hospital from 2018 to 2023

Background: In modern life, diffuse alveolar haemorrhage (DAH) is a serious and rare condition in children. Due to the subtle clinical manifestations and diverse causes of the disease, it is easy to be missed or diagnosed late. This study describes clinical and paraclinical characteristics, with the aim to find signs suggesting early diagnosis as well as determine the cause of DAH in children. Objects and methods: Case series. Results: From January 2018 to March 2023, there were 14 children diagnosed with DAH. The age of onset is 4.08 (1.58-5.41) years old, but most patients are diagnosed late with age at diagnosis being 5.25 (1.67-6.16) years old, male/female ratio is 1:1.8. The most common symptoms reported were anaemia (100%), hemoptysis (57.1%), shortness of breath (71.4%), and respiratory failure (85.7%). The common X-ray finding was diffuse infiltrates in both lungs (92.9%). On chest computed tomography scans, 92.9% of patients exhibited images of blurred opacity spreading, 57.1% showed interlobar pleural thickening, and 28.6% presented with images of lung consolidation. 3 patients (22%) had milk allergy, 2 patients (14%) had ANCA (Anti-neutrophil cytoplasmic antibody) vasculitis, and 9 patients (64%) had idiopathic pulmonary haemosiderosis (IPH).

Idiopathic Pulmonary Hemosiderosis in Pediatric in Patients: Report of Four Cases in Rabat, Morrocco

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage. Patients with IPH usually present with the classical triad: hemoptysis, and iron deficiency anemia, and pulmonary infiltrates on chest imaging, the diagnosis is often delayed by years.
 We report four IPH pediatrics cases of children diagnosed at the children’s hospital of Rabat, Morocco. The aim of this study is to review the clinical manifestations, diagnostic tools and treatment of this affection.
 All the four patients were presented with the classical triad, the diagnosis was based on the Clinical, radiologic and biologic findings. Bronchoalveolar lavage was performed to all the patients and confirmed the diagnosis by the identification of siderophages.
 High doses of Corticosteroids, by an induction therapy with an intravenous methylprednisolone (20 mg/kg/day for 3 days) followed by oral prednisone (1 mg/kg/day) had shown a good therapeutic response. One patient was treated first with oral corticosteroid and switched over to synthetic antimalarial drugs, because he had side effects from long-term cortico therapy. Then the clinical condition has been improved. The evaluation of response to the therapy included the clinical symptoms and signs, laboratory data and chest x ray. The prognosis of IPH is influenced by several factors, including the time of diagnosis, early initiation of treatment, and the presence of comorbidities.

Idiopathic pulmonary hemosiderosis as an occupational disease: a clinical case

BACKGROUND. Idiopathic pulmonary hemosiderosis (IPH) in adults is a rare pathology of unknown etiology. We did not find information about IPH in adults with occupational hazards in the available literary sources, which makes this work relevant.
 OBJECTIVE. To analyze the peculiarities of the IPH course in a patient with 23 years’ experience as a gas-electric welder at factories by our own clinical observation.
 RESULTS AND DISCUSSION. Changes in the form of heterogeneous pneumatization of the lung parenchyma due to the increased interstitial component were determined on the computer tomography of the chest cavity (CT of the chest cavity) 1.5 years before the patient’s visit, but no additional examination was prescribed, and only the diagnosis “Chronic obstructive pulmonary disease” (COPD) was made. Negative dynamics due to the increase of miliary nodular dissemination of the lungs was revealed on the CT scan of the chest after 1.5 years. IPH was already detected pathohistologically after videothoracoscopy of the right lung with biopsy. Shortness of breath and cough increased in the patient, but apart from treatment aimed at COPD treating, no other therapy was prescribed (this therapy did not have a positive effect). The patient underwent a comprehensive immunological examination: the indicators of the anti-neutrophil cytoplasmic antibodies (ANCA) profile and the complex immunological blood test were within the normal range, which ruled out pulmonary vasculitis and an immune-mediated process. Considering the positive Quantiferon test, the patient was examined and the tuberculosis was excluded. Patient have not been suffered from hemoptysis and anemia from the moment of detection of the first changes on the CT scan of chest up to the present time. Due to treatment, the clinical and radiological stabilization of the process was determined after 4 months: the clinical symptoms of the disease decreased; small nodular shadows with areas of fibrosis, which are characteristic of the period of remission, were determined on the CT scan of the chest.
 CONCLUSIONS. IPH course features in a patient with a professional route are: a long period of development with COPD manifestations (without effect of therapy), absence of hemoptysis and anemia, normal indicators of the ANCA profile and complex immunological examination, a positive Quantiferon test. Corticosteroid therapy in high doses has a positive effect, which is manifested in the clinical and radiological stabilization of the process. The mechanism of IPH development may have been a toxic mechanism (23 years of work experience as a gas-electric welder at factories). Timely detection of IPH and timely prescribed corticosteroid therapy will prevent the progression of the process and the development of complications, as well as reduce mortality from this disease. Patients with IPH should be under dynamic observation due to timely evaluation of CT-dynamics of the process in the lungs and correction of the dose of corticosteroids.

Leflunomide Is Safe and Effective for the Induction and Maintenance of Idiopathic Pulmonary Hemosiderosis Remission.

Objective: The present study was aimed to investigate the efficacy of leflunomide in idiopathic pulmonary hemosiderosis (IPH) disease control and glucocorticoid attenuation. Methods: The efficacy of leflunomide was determined based on disease control, safety, and glucocorticoid attenuation. Result: A total of 46 children with IPH were included in the present study. Of these 31 patients had been unsuccessfully treated with glucocorticoids before admission at our hospital and did not achieve complete remission; the other 15 patients had not previously received steroids. Leflunomide combined with glucocorticoid was administered to all patients, and all were followed up for a median duration of 3 years. The average hemoglobin level significantly increased and the median minimum steroid dose was significantly decreased after leflunomide administration. Conclusion: Leflunomide safely and effectively induced and maintained IPH remission and decreased IPH relapse and glucocorticoid dose.

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.

Diffuse alveolar haemorrhage in children: an international multicentre study

Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

Fatal Invasive Aspergillosis in a Child with Idiopathic Pulmonary Hemosiderosis.

Fatal Invasive Aspergillosis in a Child with Idiopathic Pulmonary Hemosiderosis.

The role of flexible bronchoscopy in the diagnostic pathway of children with unexplained peripheral eosinophilia.

Many possible underlying causes can be seen in children with unexplained peripheral eosinophilia (UPE) with suspected pulmonary involvement. Here, we aimed to demonstrate the clinical characteristics and diagnoses of children evaluated for UPE who underwent diagnostic bronchoscopy. Thirty-one children who underwent diagnostic bronchoscopy for UPE between 2006-2018 were included. Demographic characteristics, bronchoscopy findings and final diagnosis, and treatments were evaluated. The median age at the time of diagnosis was 5 (0.25-17.5) years. The final diagnoses of 26 patients were as follows: immunodeficiency (n=6); hyper-IgE syndromes (n=4), DOCK8 deficiency + HES (idiopathic hypereosinophilic syndrome; n=1), and severe combined immunodeficiency (n=1), HES (n=3), idiopathic chronic eosinophilic pneumonia (ICEP; n=3), idiopathic pulmonary hemosiderosis (n=1), B cell lymphoma (n=1). In one child, an integrin α3 mutation + cytomegalovirus (CMV) pneumonia was detected. Congenital pneumonia was found in one patient, and parasitic infection in another. In two, eosinophilia was attributed to underlying asthma and atopy. In four, the underlying reasons could not be elucidated. Two children with HES and one with ICEP were lost to follow-up. There was no significant relationship between the peripheral eosinophil count at the time of diagnosis and the percentage of bronchoalveolar lavage eosinophils (BAL). Bronchoscopy contributed to the management of 14 (53.9%) patients. Bronchoscopy has potential diagnostic contribution in patients with UPE suspected of having pulmonary involvement. Numerous various underlying causes may be present in this patient group. There is no relation between peripheral eosinophil count and BAL eosinophil percentage.

A case report Case Report of Idiopathic Pulmonary Haemosiderosis in a Child with recurrent chest infections

Idiopathic Pulmonary Hemosiderosis (IPH) is a rare condition, usually occurring in children and teenagers. The classic presentation is of haemoptysis, iron deficiency anaemia and diffuse pulmonary infiltrates. We report a case of IPH in an 8 year old boy, who presented with recurrent episodes of fever, cough and respiratory infections without any trigger since childhood.

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

This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhagein children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinicaland 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 dietcan manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroidsare 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 end-stage lung disease despite all measures, and lung transplantation may be the only viable option.

Assessment of type I interferon signatures in undifferentiated inflammatory diseases: A Japanese multicenter experience.

PurposeUpregulation of type I interferon (IFN) signaling has been increasingly detected in inflammatory diseases. Recently, upregulation of the IFN signature has been suggested as a potential biomarker of IFN-driven inflammatory diseases. Yet, it remains unclear to what extent type I IFN is involved in the pathogenesis of undifferentiated inflammatory diseases. This study aimed to quantify the type I IFN signature in clinically undiagnosed patients and assess clinical characteristics in those with a high IFN signature.MethodsThe type I IFN signature was measured in patients’ whole blood cells. Clinical and biological data were collected retrospectively, and an intensive genetic analysis was performed in undiagnosed patients with a high IFN signature.ResultsA total of 117 samples from 94 patients with inflammatory diseases, including 37 undiagnosed cases, were analyzed. Increased IFN signaling was observed in 19 undiagnosed patients, with 10 exhibiting clinical features commonly found in type I interferonopathies. Skin manifestations, observed in eight patients, were macroscopically and histologically similar to those found in proteasome-associated autoinflammatory syndrome. Genetic analysis identified novel mutations in the PSMB8 gene of one patient, and rare variants of unknown significance in genes linked to type I IFN signaling in four patients. A JAK inhibitor effectively treated the patient with the PSMB8 mutations. Patients with clinically quiescent idiopathic pulmonary hemosiderosis and A20 haploinsufficiency showed enhanced IFN signaling.ConclusionsHalf of the patients examined in this study, with undifferentiated inflammatory diseases, clinically quiescent A20 haploinsufficiency, or idiopathic pulmonary hemosiderosis, had an elevated type I IFN signature.

Influence of liquids for electronic cigarettes on the human respiratory system. Clinical observation of a patient with EVALI

Aim. Demonstration of clinical observation of EVALI from the personal practice of the authors.Materials and methods. A brief review of the literature on the effects of e-liquids on the human respiratory system is presented. Clinical observation of this pathology is given.Results. Patient H., 19 years old, was admitted to the Thoracic Surgery Department with complaints of shortness of breath, cough with hemoptysis. On computer tomography in lung parenchyma, infiltrates are found in all fields with extensive ground glass areas. Fibrobronchoscopy revealed in the lumen of the bronchi a small amount of hemorrhagic “crusts” and hemorrhagic exudate, catarrhal-hemorrhagic endobronchitis. The patient is a fan of electronic cigarettes, which was not immediately paid attention to. A differential diagnosis was made with Goodpasture's syndrome, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus with lung involvement, and ANCA-associated vasculitis. Since there was nephropathy and there were negative immunological findings of other diseases, a diagnosis of Goodpasture's syndrome was made. A kidney biopsy was not performed. The rapid positive dynamics from hormonal therapy and the absence of relapses and progression raised doubts about this diagnosis. After the disease, the patient refused to take electronic cigarettes. The anamnesis was again addressed, where there were indications of the abuse of electronic cigarettes, after frequent use of which a detailed clinical picture of the disease appeared, the diagnosis of Goodpasture's syndrome was withdrawn, lung damage was regarded as a manifestation of EVALI.Conclusion. Confirmation of the negative impact of electronic smoking systems is the described clinical case, indicating lung damage in humans and difficulties in the differential diagnosis of EVALI syndrome.

Recurrence of idiopathic pulmonary hemosiderosis in adults with childhood onset: A case report and literature review

We describe a rare case of a 20-year-old Japanese man with idiopathic pulmonary hemosiderosis (IPH) recurrence in adults with childhood onset (racIPH). IPH commonly occurs in children, and data regarding racIPH are lacking. A review of the literature showed that only five cases of racIPH have been reported (including the present case) and that racIPH shows features that are intermediate between childhood- and adult-onset IPH with respect to age and a lower frequency of smoking history. We also found that the degree of anemia was usually not severe, and a favorable response to corticosteroid therapy is expected in racIPH.

The Spectrum of Autoantibodies in Adult Patients With Idiopathic Pulmonary Hemosiderosis: A Brief Review of the Literature.

While autoimmune antibodies or autoantibodies have been reported sporadically in adult patients with idiopathic pulmonary hemosiderosis (IPH), their true prevalence is unknown. The question as to whether any difference exists between antibody-positive and negative patients has not been explored. The primary objective of this paper was to assess the spectrum of autoantibody testing and its positivity rate. The other objectives included a comparative analysis of demographics, symptom onset, clinical manifestations, and differences in clinical outcomes between antibody-positive (cohort A) and negative (cohort B) patients. To that end, we conducted a retrospective review of the relevant published literature. Multiple databases were searched to retrieve studies published between 1990 and 2022.A total of 35 studies, involving 38 patients, were identified. Five of these patients had a positive autoantibody. Patients in cohort A were older and more likely to be male. The frequencies of testing for these antibodies were as follows: antineutrophil cytoplasmic antibody (ANCA): 37/38 (97.4%), antinuclear antibody (ANA): 31/38 (81.6%), and anti-glomerular basement membrane antibody (anti-GBM): 30/38 (78.9%); 5/38 (13.2%) patients tested positive for an autoantibody, and two of these patients were positive for ANA, two for antithyroid antibody, and one patient tested positive for ANCA, rheumatoid factor (RF), and granulocyte monocyte-colony stimulating factor (GM-CSF) antibody. There was no difference between the cohorts regarding their clinical presentations, recurrence risks, and survival.The occurrence of autoantibodies is uncommon in adult IPH patients. This is in contrast with the pediatric IPH patient population, where the prevalence is much higher (26.4% vs. 13.2%), and the antibodies are more diverse. Unlike pediatric patients, adult patients with autoantibodies do not necessarily have worse outcomes.

Comparative Analysis of Adult Patients With Idiopathic Pulmonary Hemosiderosis and Lane-Hamilton Syndrome: A Systematic Review of the Literature in the Period 1971-2022.

Idiopathic pulmonary hemosiderosis (IPH) causes diffuse alveolar hemorrhage (DAH) by a yet unknown mechanism. The coexistence of IPH and celiac disease (CD), also known as Lane-Hamilton syndrome (LHS), has been reported in both pediatric and adult patients. The objective of this study was to compare demographics, clinical and radiologic findings, treatment, and outcomes between adult patients with IPH and LHS.This is a systematic review of the literature. Multiple databases were searched using appropriate formulas to identify relevant articles. A total of 60 studies reporting 65 patients were included in the review. Forty-nine of these patients had IPH and 16 had LHS. The prevalence of anti-CD antibodies among tested patients was 13/22 (59%). The symptom onset and diagnosis of IPH occurred earlier in patients with LHS. The median delay in diagnosis was the same between the two groups (52 weeks). The classic triad was more likely to be present in patients with LHS. Only 20% of patients in the LHS cohort had any significant gastrointestinal (GI) symptoms at the time of IPH diagnosis. A gluten-free diet alone was effective in the majority of patients. Fewer patients in the LHS cohort received systemic corticosteroid than the IPH cohort. The recurrence and mortality in patients with LHS appear to be less than in the IPH cohort. The prevalence of CD is 25% in adult patients with IPH. Patients with LHS may have a milder course than patients without CD. Serologic testing for CD should be performed in all patients diagnosed with IPH.

Proposed Pathogenesis of Diffuse Alveolar Hemorrhage in Idiopathic Pulmonary Hemosiderosis.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease that causes diffuse alveolar hemorrhage (DAH). The latest data suggests an immunologic origin of IPH, and a new name, immune mediated pulmonary hemosiderosis (ImPH), has been proposed. However, the exact immunologic mechanism has remained elusive for nearly eight decades despite extensive research, including detailed histopathologic analysis. Although several hypotheses have been proposed to describe the pathobiology of IPH, none of them explain the clinical and histopathologic findings conclusively. In this manuscript, we have presented a new hypothesis for the pathogenesis of DAH in IPH. We hypothesize that DAH in IPH is not immunocomplex mediated but due to histamine, eosinophilic cationic protein (ECP), and possibly vascular endothelial growth factor (VEGF). These bioactive proteins induce endothelial and alveolar epithelial damage, leading to the peri-capillary and intraalveolar escape of RBCs. The deformability of the RBC likely also plays a role. The supranormal secretion of histamine, ECP and VEGF occurs in genetically predisposed individuals with an aberrant immunologic response. The histamine is released from the basophils and possibly the mast cells in response to cytokines secreted by activated lymphocytes. The lymphocyte activation occurs after exposure to a known (gluten) or unknown antigen. The same lymphocyte-derived cytokines also induce eosinophilic degranulation of ECP and VEGF in the pulmonary circulation. We believe that our hypothesis unifies the observed clinical variabilities and histopathologic findings in IPH, and we hope that would promote future research in the field of IPH.

Appreciation to Reviewers in 2021

We describe a rare case of a 20-year-old Japanese man with idiopathic pulmonary hemosiderosis (IPH) recurrence in adults with childhood onset (racIPH). IPH commonly occurs in children, and data regarding racIPH are lacking. A review of the literature showed that only five cases of racIPH have been reported (including the present case) and that racIPH shows features that are intermediate between childhood- and adult-onset IPH with respect to age and a lower frequency of smoking history. We also found that the degree of anemia was usually not severe, and a favorable response to corticosteroid therapy is expected in racIPH.

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.