scholarly journals Idiopathic Pulmonary Hemosiderosis in an Adult with Unusual Radiologic Features

2014 ◽  
Vol 48 (4) ◽  
pp. 199-200
Author(s):  
Sangeeth Kumar Kasilingam ◽  
Shylaja Prashanth ◽  
Manjunath B Govindagoudar ◽  
Bheemaraya Devaramani ◽  
Chaitra Channarayapatna Swamygowda

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.

2020 ◽  
Vol 90 (2) ◽  
Author(s):  
Ayesha Butt ◽  
Rashida Ahmed ◽  
Muhammad Dawood Amir Sheikh ◽  
Omar Khan ◽  
Nousheen Iqbal ◽  
...  

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.


2019 ◽  
Vol 08 (03) ◽  
pp. 181-186 ◽  
Author(s):  
Shotaro Matsumoto ◽  
Satoshi Nakagawa

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.


2016 ◽  
Vol 25 (4) ◽  
pp. 555-558
Author(s):  
Alina Popp

Background: Alveolar hemorrhage is a potentially life-threatening condition which is usually managed by the pulmonologist. When considering its etiology, there is a rare association that sets the disease into the hands of the gastroenterologist. Case presentation: We report the case of a 48 year-old female who was admitted to the intensive care unit for severe anemia and hemoptysis. On imaging, diffuse pulmonary infiltrates suggestive of alveolar hemorrhage were detected and a diagnosis of pulmonary hemosiderosis was made. She received cortisone therapy and hematologic correction of anemia, with slow recovery. In search of an etiology for the pulmonary hemosiderosis, an extensive workup was done, and celiac disease specific serology was found positive. After confirmation of celiac disease by biopsy, a diagnosis of Lane-Hamilton syndrome was established. The patient was recommended a gluten-free diet and at 6 months follow-up, resolution of anemia and pulmonary infiltrates were observed. Conclusion: Although the association is rare, celiac disease should be considered in a patient with idiopathic pulmonary hemosiderosis. In our case, severe anemia and alveolar infiltrates markedly improved with glucocorticoids and gluten-free diet. Abbreviations: APTT: activated partial thromboplastin time; BAL: bronchoalveolar lavage; CD: celiac disease; Cd: crypt depth; GFD: gluten-free diet; GI: gastrointestinal; IEL: intraepithelial lymphocyte; INR: international normalized ratio; IPH: idiopathic pu


2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Boris Limme ◽  
Ramona Nicolescu ◽  
Jean-Paul Misson

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.


2020 ◽  
pp. 72-80
Author(s):  
O.L. Tsymbalista ◽  

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.


2013 ◽  
Vol 8 ◽  
Author(s):  
Kamal Kumar Singhal ◽  
Ashok K. Janmeja ◽  
Rakhee Sodhi ◽  
Rajpal S. Punia

Idiopathic Pulmonary Hemosiderosis (IPH) is characterized by the triad of iron deficiency anemia, pulmonary infiltrates and haemoptysis with no recognizable cause. Since the first description of its association with Celiac Disease (CD) by Lane and Hamilton in 1971, only a few isolated cases have been reported in literature. Although it has been considered an uncommon association of two disease entities, recent reports indicate that prevalence of celiac disease is as high as one percent. Further, individually both celiac disease and IPH are known to present as refractory anemia only. We are reporting a young adult with Lane Hamilton Syndrome, who realized that he was having significant gastrointestinal complaints only when they disappeared on gluten free diet (GFD). This case report reiterates the fact that celiac disease should be considered in all patients of IPH because of the therapeutic implications. Further on review of literature, we believe that covert hemoptysis may be responsible for disproportionately severe anemia in patients of celiac disease. Thus, prevalence of this association may be more than currently believed. Further research in this regard may improve our understanding of pathogenesis of celiac disease.


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