Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach

ABSTRACT Idiopathic pulmonary hemosiderosis is an exceptionally rare cause of diffuse alveolar hemorrhage that occurs primarily in infants and children. Few cases are reported in adults. Patients usually present with recurrent episodes of hemoptysis, fatigue breathlessness and severe anemia with asymptomatic periods in between. Our patient, who was diagnosed with idiopathic pulmonary hemosiderosis demonstrated diffuse ground glass opacity, focal centrilobular emphysema and multiple sub pleural cysts and few parenchymal cysts during acute episode of hemoptysis. Unusual findings in our case were sub pleural cysts, parenchymal cysts and focal centrilobular emphysema which was not documented in any case reports of idiopathic pulmonary hemosiderosis cases to that of our knowledge. How to cite this article Kasilingam SK, Prashanth S, Govindagoudar MB, Devaramani B, Swamygowda CC. Idiopathic Pulmonary Hemosiderosis in an Adult with Unusual Radiologic Features. J Postgrad Med Edu Res 2014;48(4):199-200.

Download Full-text

Extracorporeal Membrane Oxygenation for Diffuse Alveolar Hemorrhage Caused by Idiopathic Pulmonary Hemosiderosis: A Case Report and a Review of the Literature

Journal of Pediatric Intensive Care ◽

10.1055/s-0039-1679904 ◽

2019 ◽

Vol 08 (03) ◽

pp. 181-186 ◽

Cited By ~ 2

Author(s):

Shotaro Matsumoto ◽

Satoshi Nakagawa

Keyword(s):

Extracorporeal Membrane Oxygenation ◽

Rescue Therapy ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Membrane Oxygenation ◽

Pulmonary Hemosiderosis ◽

Threatening Condition ◽

Life Threatening ◽

Systemic Anticoagulation ◽

Idiopathic Pulmonary Hemosiderosis

AbstractDiffuse alveolar hemorrhage (DAH) is a life-threatening condition presenting with hemoptysis, anemia, and diffuse radiographic pulmonary infiltrates; it causes acute respiratory failure. Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of DAH occurring predominantly in children. Bleeding is often considered to be a contraindication for extracorporeal membrane oxygenation (ECMO) due to systemic anticoagulation. We present an 8-year-old girl with DAH caused by IPH. Unfractionated heparin was administered to maintain an activated clotting time of 150 to 180 seconds. The DAH resolved with immunosuppressive therapy, and the patient survived to decannulation. ECMO may be applied as a rescue therapy for DAH even with systemic anticoagulation.

Download Full-text

Idiopathic Pulmonary Hemosiderosis – a Severe Differential Diagnosis of Diffuse Alveolar Hemorrhage

Klinische Pädiatrie ◽

10.1055/s-0031-1287819 ◽

2011 ◽

Vol 223 (06) ◽

pp. 376-377

Author(s):

F. Koerber ◽

E. Rietschel ◽

M. Feldkötter

Keyword(s):

Differential Diagnosis ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Pulmonary Hemosiderosis ◽

Idiopathic Pulmonary Hemosiderosis

Download Full-text

Diffuse Alveolar Hemorrhage in Autoimmune Diseases

Current Rheumatology Reports ◽

10.1007/s11926-017-0651-y ◽

2017 ◽

Vol 19 (5) ◽

Cited By ~ 11

Author(s):

Marco Ulises Martínez-Martínez ◽

David Alejandro Herrera-van Oostdam ◽

Carlos Abud-Mendoza

Keyword(s):

Autoimmune Diseases ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage

Download Full-text

Neonatal Pulmonary Hemosiderosis

Case Reports in Pediatrics ◽

10.1155/2014/463973 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

Boris Limme ◽

Ramona Nicolescu ◽

Jean-Paul Misson

Keyword(s):

Emergency Department ◽

Respiratory Distress ◽

Bronchoalveolar Lavage ◽

Immunosuppressive Therapy ◽

Clinical Presentation ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Type ◽

Histological Studies ◽

Pulmonary Hemosiderosis ◽

Idiopathic 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.

Download Full-text

Intra-Alveolar Hemorrhage in Certain Orphan Lung Diseases in Children (Lecture)

Modern pediatrics. Ukraine ◽

10.15574/sp.2020.111.72 ◽

2020 ◽

pp. 72-80

Author(s):

O.L. Tsymbalista ◽

Keyword(s):

Kidney Failure ◽

Clinical Course ◽

Pulmonary Hemorrhage ◽

Alveolar Hemorrhage ◽

Rapid Progression ◽

Goodpasture Syndrome ◽

Pulmonary Hemosiderosis ◽

Short Period ◽

Pulmonary Pathology ◽

Idiopathic Pulmonary Hemosiderosis

The theme is relevant due to the diagnostic difficulties, severe clinical course and prognosis of idiopathic pulmonary hemosiderosis and Goodpasture syndrome. Idiopathic pulmonary hemosiderosis and Goodpasture syndrome are severe, life-threatening immunopathologic diseases due to alveolar hemorrhage and a hundred percent mortality within a short period of time after the onset of clinical manifestations. Idiopathic pulmonary hemosiderosis generally occurs in children at the age of 3–8 years as a separate condition, or as a stage of Goodpasture syndrome. It manifests itself as shortness of breath, pneumonia, prune juice sputum, hemoptysis, hemorrhage. During exacerbation, the patients' condition is determined by the degree of pulmonary hemorrhage, pulmonary heart disease, acute posthemorrhagic anemia. The exacerbation lasts from a few hours to 1–2 weeks. The duration of each episode and remission varies among patients being unpredictable. Each new exacerbation is more severe. In Goodpasture syndrome, predominant pulmonary and renal vascular lesions of autoimmune nature are observed. It affects young males more frequently; is rare in children. Hemorrhagic alveolitis as a form of lung damage develops first; then, the kidneys are involved, and anemia occurs. Glomerulonephritis (GN) manifests itself as nephrotic syndrome with rapid progression of kidney failure. In case of the predominant pulmonary pathology, recurrent hemoptysis and pulmonary hemorrhage are observed; in end-stage disease with cardiopulmonary failure manifestations, rapidly progressive GN and kidney failure develop. The second variant of Goodpasture syndrome is characterized by relatively slow progression of pulmonary changes and renal lesions. Goodpasture syndrome is rarely accompanied by GN from the onset to the end of the disease and pulmonary pathology manifests itself at the terminal phase of the disease. The treatment of both diseases includes lifetime therapy with glucocorticoids, cytostatics. Pulse therapy using these preparations, discrete plasma exchange and intravenous immunoglobulin administration, syndromic treatment are carried out. No conflict of interest was declared by the authors. Keywords: children, idiopathic pulmonary hemosiderosis, Goodpasture syndrome, clinical course, therapy.

Download Full-text

Idiopathic pulmonary hemosiderosis - A rare cause of chronic anemia

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2020.1267 ◽

2020 ◽

Vol 90 (2) ◽

Cited By ~ 1

Author(s):

Ayesha Butt ◽

Rashida Ahmed ◽

Muhammad Dawood Amir Sheikh ◽

Omar Khan ◽

Nousheen Iqbal ◽

...

Keyword(s):

Lung Biopsy ◽

Diffuse Alveolar Hemorrhage ◽

Severe Anemia ◽

Chronic Anemia ◽

Posterior Reversible Encephalopathy ◽

The Third ◽

First Case ◽

Pulmonary Hemosiderosis ◽

Reversible Encephalopathy ◽

Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease marked by alveolar bleeding and accumulation of hemosiderin in the lungs. Here we present three cases of IPH. The first case is of a 26-year-old male with anemia, hemoptysis and dyspnea. Bronchoscopy confirmed diffuse alveolar hemorrhage (DAH). A diagnosis of IPH was made after ruling out other causes of DAH and observing good response to steroids. The patient’s condition improved with prednisolone and azathioprine. The second case is of 26-year-old female with severe anemia. Imaging suggested IPH and lung biopsy confirmed it. She died shortly afterwards. The third case is of a 7-year-old male with chronic anemia. CT was suggestive of IPH and lung biopsy confirmed the diagnosis. Later, patient developed posterior reversible encephalopathy syndrome (PRES). This patient is stable on azathioprine and prednisolone. We aim to emphasize the importance of considering IPH as a differential in patients with DAH or chronic anemia.

Download Full-text

IDIOPATHIC PULMONARY HEMOSIDEROSIS AS A DIFFERENTIAL DIAGNOSIS OF AUTOIMMUNE DISEASES: CASE REPORT

10.47660/cbr.2020.17292 ◽

2021 ◽

Author(s):

Danielly Dantas Pimentel ◽

Gizelle Gouvea Rezende ◽

Gustavo Roberto Lourenço ◽

Iane Tamara Dondé ◽

Juliana de Jesus Boscolo ◽

...

Keyword(s):

Differential Diagnosis ◽

Case Report ◽

Autoimmune Diseases ◽

Pulmonary Hemosiderosis ◽

Idiopathic Pulmonary Hemosiderosis

Download Full-text

Diffuse Alveolar Hemorrhage in Children with Trisomy 21

10.21203/rs.3.rs-297512/v1 ◽

2021 ◽

Author(s):

Jessica L. Bloom ◽

Benjamin Frank ◽

Jason P. Weinman ◽

Csaba Galambos ◽

Sean T. O’Leary ◽

...

Keyword(s):

Respiratory Distress ◽

Trisomy 21 ◽

Immune Modulation ◽

The United States ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Nuclear Antigen ◽

Recurrent Pneumonia ◽

Pulmonary Hemosiderosis ◽

Common Diagnosis

Abstract Background: Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case Presentation: Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for ten years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. A minority had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation.Conclusion: These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

Download Full-text

Diffuse alveolar hemorrhage in children with trisomy 21

Pediatric Rheumatology ◽

10.1186/s12969-021-00592-4 ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Jessica L. Bloom ◽

Benjamin Frank ◽

Jason P. Weinman ◽

Csaba Galambos ◽

Sean T. O’Leary ◽

...

Keyword(s):

Respiratory Distress ◽

Trisomy 21 ◽

Immune Modulation ◽

The United States ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Nuclear Antigen ◽

Recurrent Pneumonia ◽

Pulmonary Hemosiderosis ◽

Common Diagnosis

Abstract Background Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case presentation Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for 10 years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. Very few patients had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation. Conclusion These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, particularly given the low frequency of hemoptysis at presentation, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

Download Full-text