Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Visit old site
Home Print this page Email this page Small font size Default font size Increase font size
Users Online: 1428

 Table of Contents  
Year : 2012  |  Volume : 4  |  Issue : 1  |  Page : 49-51

Eight years follow-up of a case with idiopathic pulmonary hemosiderosis after corticosteroid therapy

1 Department of Chest Diseases, Kahramanmaras Sutcuimam University, Kahramanmaras, Turkey
2 Department of Radiology, Kahramanmaras Sutcuimam University, Kahramanmaras, Turkey

Date of Web Publication13-Feb-2012

Correspondence Address:
Hasan Kahraman
Department of Chest Diseases, Kahramanmaras Sutcu Imam University, Yörükselim Mah. Hastane Cad. No: 32, Kahramanmaras
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1947-2714.92907

Rights and Permissions

Idiopathic pulmonary hemosiderosis is characterized by recurrent episodes of alveolar hemorrhage, hemoptysis, and secondary iron deficiency anemia with unknown etiology. It generally emerges in childhood and adolescence periods but rarely found in adulthood. Definite diagnosis is established by appearing the hemosiderin-laden macrophages at sputum, bronchoalveolar lavage, or open lung biopsy. We reported a male patient who was born in 1975, expectorated blood since 1995, and was diagnosed in 1998. He received many blood replacements. He admitted to our clinic in 2003 with complaints of coughing up blood, shortness of breath, and tiredness. We gave the corticosteroid therapy to patient for 6-month period. After treatment, the patient did not have any complaints. Clinicians should keep in mind that idiopathic pulmonary hemosiderosis may differ in localization on chest X-ray and corticosteroid treatment should be started when diagnosis is established.

Keywords: Alveolar hemorrhage, Corticosteroid therapy, Pulmonary hemosiderosis

How to cite this article:
Kahraman H, Köksal N, Özkan F. Eight years follow-up of a case with idiopathic pulmonary hemosiderosis after corticosteroid therapy. North Am J Med Sci 2012;4:49-51

How to cite this URL:
Kahraman H, Köksal N, Özkan F. Eight years follow-up of a case with idiopathic pulmonary hemosiderosis after corticosteroid therapy. North Am J Med Sci [serial online] 2012 [cited 2023 Feb 5];4:49-51. Available from: https://www.najms.org/text.asp?2012/4/1/49/92907

  Introduction Top

Idiopathic pulmonary haemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) with unknown etiology. [1] DAH is characterized by haemoptysis, dyspnea, alveolar infiltrates on chest X-ray, and various degrees of anemia. In IPH, intra-alveolar bleeding is persistent or recurrent, and often marked. [2],[3] It is classic disease of childhood, but approximately 20% of patients are presented during adulthood. Radiographical manifestations of IPH include patchy areas of opacities that usually involve both lungs and opacities similar to pulmonary edema. The costophrenic angles and periphery of the lungs are usually spared. Absolute diagnosis is based on the presence of hemosiderin-laden macrophages (HLM) without any evidence of pulmonary vasculitis, nonspecific granulomatous inflammation or deposition of immunoglobulins in respiratory secretion or biopsy materials. [1] We reported the young adult man case because of 16-year period of IPH disease; responding to corticosteroid treatment very well and 8-year follow up after treatment.

  Case Report Top

A 29-year old man was admitted to our clinic with complaints of fatigue, pallor, cough, bloody sputum, and shortness of breath in 2003. In history, his complaints began in 1995 and HRCT was taken in 1998. It showed a decrease in aeration and patchy areas of ground glass pattern in both lungs, especially in the lower lobes. Open lung biopsies were taken by video-assisted thorascopic technique. In pathology, a large number of HLM, interstitial edema and alveolar fibrosis, and foci of fresh hemorrhage were determined and he was diagnosed as idiopathic pulmonary hemosiderosis [Figure 1]. Patient's hemoptysis continued for years. In 2003, he was hospitalized. After blood transfusions, peripheral white cell count (WBC) and platelet (PLT) count were normal, hemoglobin (Hb) was 5.3 g/dl. Serum biochemistry, ANA, p-ANCA, C-ANCA, and RF were normal. Erythrocyte sedimentation rate (ERS) was 28 mm/h, serum iron was 18 mg/dl, iron binding capacity was 300 mg/dl. Electrocardiography was normal, arterial blood gases were as follows: pH 7.4, PO2 66, PCO2 38, SaO2 94%.
Figure 1: A large number of hemosiderin-laden macrophages and foci of fresh hemorrhage were determined. H and E, ×100 and Prussian blue, ×100

Click here to view

Two month later hemoptysis repeated and patient was admitted to our clinic. His and family history was not contributory. He had an 11-pack-year smoking history. Physical examination revealed pale skin and mucosa, other vital signs were normal. In respiratory system examination bilateral basal crackles were heard. Cardiovascular, gastrointestinal, and neurological examinations were normal.

A daily dose of 80 mg (1.5 mg/kg) methylprednisolone was started, tapered to 48 mg/day 1 month later, and was stopped at the end of 6th month. The patient responded to treatment very well, and after that he had no complains. We invited him to control in 2009 and HRCT was given. Peripheral ground-glass appearance at the upper and middle lobe of right lung, and at the upper lobe of left lung were determined [Figure 2]. Hb 13.2 g/dl, WBC, PLT, and ESR were normal and pulmonary function tests showed mild restriction. It has been 16 years from the begining of hemoptysis, 13 years from the diagnosis and 8 years from the treatment. Since the beginning of treatment, there has been a very small amount of hemoptysis approximately less than 1 cc 1-2 times per year.
Figure 2: Peripheral ground-glass appearance at the upper and middle lobe of right lung, and at the upper lobe of left lung

Click here to view

  Discussion Top

Idiopathic pulmonary hemosiderosis is a rare disorder of unknown etiology and usually appears in children. [3] The etiology of IPH has been suspected to be autoimmune, allergic, genetic or environmental in different theories. [4] Some reports presenting that gluten-sensitive enteropathy and sensitivity to cow's milk may accompany IPH. [5] Our case was a young adult and such situations were not detected in our patients.

Findings of hemoptysis, iron deficiency anemia, shortness of breath, and parenchymal infiltration in chest radiography are typical as seen in many clinical articles. [6] Our patient had typical findings too.

HRCT appearances include patchy scattered areas of ground glass opacity and consolidation that usually involve the hilary and lower regions of the lungs, but involvement may be diffused. [7] Lymphadenopathy and pleural effusions are not common. [8] Interstitial fibrosis was developed in our patient. But in our case peripheral ground-glass appearance was presented at upper and middle lobe of right lung, and at the upper lobe of left lung. It is different from general articles.

For definitive diagnosis, lung biopsy is necessary, the most important features are the presence of intact erythrocytes and numerous siderophages in the alveoli and the absence of any other pathologic sign like vascular malformation, immune deposition, capillaritis, vasculitis, granuloma, malignancy. [3] In our patient, IPH was diagnosed with the finding of HLM in lung biopsy material as in other studies. [1],[9]

The use of systemic glucocorticoids for IPH grew out of the impression of an immune pathogenesis. Although the disease is rare, information from case reports and case series suggest that systemic glucocorticoids reduce the morbidity and mortality of acute episodes of alveolar bleeding and control progression to pulmonary fibrosis. [3],[6],[10] The recommended dose was <1 mg/kg/day prednisolone for two months period and then gradually reduced. [1] In one study, methylprednisolone in dose of 1-2 mg/kg/day was suggested. [8] We started daily dose of 80 mg (1.5 mg/kg) treatment and tapered to 48 mg/day 1 month later, and stopped at end of 6th month. The patient responded the treatment very well, as in other studies. [6],[10]

In conclusion, we think that during childhood and young adult periods IPH should be considered in patients presenting hemoptysis and anemia. For diagnosis tissue samples should be taken and as a treatment systemic corticosteroid therapy should be given.

  Acknowledgment Top

We thank to Pathology Department of Gülhane Military Medical Academy for sending pathology slide photographs.

  References Top

1.Gencer M, Ceylan E, Bitiren M, Koc A. Two sisters with idiopathic pulmonary hemosiderosis. Can Respir J 2007;14:490-3.  Back to cited text no. 1
2.Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J 2004;24:162-70.  Back to cited text no. 2
3.Green RJ, Ruoss SJ, Kraft SA, Duncan SR, Berry GJ, Raffin TA. Pulmonary capillaritis and alveolar haemorrhage. Update on diagnosis and management. Chest 1996;110:1305-16.  Back to cited text no. 3
4.Agata H, Kondo N, Fukutomi O, Takemura M, Tashita H, Kobayashi Y, et al. Pulmonary hemosiderosis with hypersensitivity to buckwheat. Ann Allergy Asthma Immunol 1997;78:233-7.  Back to cited text no. 4
5.Morgan PG, Turner-Warwick M. Pulmonary haemosiderosis and pulmonary haemorrhage. Br J Dis Chest 1981;75:225-42.   Back to cited text no. 5
6.Soergel K, Sommers SC. Idiopathic pulmonary hemosiderosis and related syndromes. Am J Med 1962;32:499-511.  Back to cited text no. 6
7.Harte S, McNicholas WT, Donnelly SC, Dodd JD. Honeycomb cysts in idiopathic pulmonary haemosiderosis: High-resolution CT appearances in two adults. Br J Radiol 2008;81:295-8.  Back to cited text no. 7
8.Abbdallah Fatma CB, Amel C, Ridha M, Olfa S, Sophia T, Faouzi M, et al. Idiopathic pulmonary hemosiderosis in adult. Respir Med CME 2010;3:238-40.  Back to cited text no. 8
9.Erdað GÇ, Vitrinel A, Yeþiltepe Mutlu G, Akýn Y, Sadýkoðlu S. Idiopathic pulmonary hemosiderosis: A case report. Marmara Med J 2006;19:33-5.  Back to cited text no. 9
10.Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol D. Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): Prolonged survival with low-dose corticosteroid therapy. Pediatr Pulmonol 1999;27:180-4.  Back to cited text no. 10


  [Figure 1], [Figure 2]

This article has been cited by
1 Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature
Biplab K. Saha, Alyssa Bonnier, Santu Saha, Baidya Nath Saha, Boris Shkolnik
Clinical Rheumatology. 2022;
[Pubmed] | [DOI]
2 Comparative Analysis of Adult Patients With Idiopathic Pulmonary Hemosiderosis and Lane-Hamilton Syndrome: A Systematic Review of the Literature in the Period 1971-2022
Biplab K Saha, Praveen Datar, Alexis Aiman, Alyssa Bonnier, Santu Saha, Nils T Milman
Cureus. 2022;
[Pubmed] | [DOI]
3 Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome
Hiromi Tobai,Jun Yano,Norio Sato,Fumitaka Amanuma,Mikio Takahashi,Mikiya Endo,Masataka Ishimura,Shouichi Ohga,Hidekazu Maruyama
Case Reports in Pediatrics. 2020; 2020: 1
[Pubmed] | [DOI]
4 A Case of Idiopathic Pulmonary Hemosiderosis Presenting With Signs and Symptoms Mimicking Hemolytic Anemia
Dilek Dogruel,Ayse Erbay,Nalan Yazici,Alev Arslan,Bermal Hasbay Biçen
Journal of Pediatric Hematology/Oncology. 2017; 39(1): e15
[Pubmed] | [DOI]
5 Interstitial lung diseases - Can pathologists arrive at an etiology-based diagnosis? A critical update
Popper, H.H.
Virchows Archiv. 2013; 462(1): 1-26
6 Interstitial lung diseases—can pathologists arrive at an etiology-based diagnosis? A critical update
Helmut H. Popper
Virchows Archiv. 2013; 462(1): 1
[Pubmed] | [DOI]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded479    
    Comments [Add]    
    Cited by others 6    

Recommend this journal