|Year : 2018 | Volume
| Issue : 1 | Page : 38-40
Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case
Abhishek Agarwal1, Rahul Kumar2, Anand Jaiswal1, Vikram Vohra1, Poulomi Chatterjee2
1 Department of Tuberculosis and Chest Diseases, National Institute of Tuberculosis and Respiratory Diseases, New Delhi, India
2 Department of Respiratory and Sleep Medicine, Medanta − The Medicity, Gurugram, Haryana, India
|Date of Web Publication||3-Jan-2018|
Department of Respiratory and Sleep Medicine, Medanta – The Medicity, Gurugram, Haryana
Source of Support: None, Conflict of Interest: None
Idiopathic pulmonary hemosiderosis (IPH) is often an ignored and rare cause of diffuse alveolar hemorrhage (DAH). It is characterized by triad of hemoptysis, anemia, and alveolar opacity on radiology. It is a diagnosis of exclusion, established after ruling out other causes of DAH such as Goodpasture’s syndrome, large vessel vasculitis, small vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) (Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis), immune complex-related vasculitis (collagen vascular diseases, Henoch–Schönlein purpura, mixed cryoglobulinemia) drug reactions, anticoagulation and thrombocytopenia. Though it is a disease primarily affecting children, we hereby report a case of IPH in an adult patient who responded dramatically to oral corticosteroid.
Keywords: Corticosteroid, diffuse alveolar hemorrhage, idiopathic pulmonary hemosiderosis
|How to cite this article:|
Agarwal A, Kumar R, Jaiswal A, Vohra V, Chatterjee P. Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case. J Assoc Chest Physicians 2018;6:38-40
|How to cite this URL:|
Agarwal A, Kumar R, Jaiswal A, Vohra V, Chatterjee P. Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case. J Assoc Chest Physicians [serial online] 2018 [cited 2019 Nov 14];6:38-40. Available from: http://www.jacpjournal.org/text.asp?2018/6/1/38/217314
| Introduction|| |
Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) with unknown etiology. Though it primarily affects children, adult cases have also been reported. The exact incidence and prevalence of IPH are not yet known. The estimated incidence is 0.2–1.2 cases per million. Usually such patients present with recurrent episodes of hemoptysis, anemia, and dyspnea. It is a diagnosis of exclusion established by ruling out other causes of DAH.
| Case History|| |
A 27-year-old male student presented with chief complaints of recurrent hemoptysis for 3 years and exertional breathlessness for 3 years. He had no history of chest pain, abdominal pain, palpitation, fever, weight loss, night sweat, skin rash, joint pain, mucosal ulcer, redness of eyes, hematuria, and loss of appetite. He is a non-smoker, no history of illicit-drug abuse. He had empirically received antitubercular treatment, but was not relieved of symptoms. Besides antitubercular treatment, this patient has not taken any other medication. On examination, a moderate pallor was present, pulse rate was 100 beats/min, blood pressure was 110/70 mmHg in right upper limb, and respiratory rate was 24 breaths/min. There was no clubbing, no cyanosis, no icterus, no lymphadenopathy. Chest examination revealed bilateral fine end inspiratory crackles. Examination of rest of the organ system was normal. His blood investigation revealed iron deficiency anemia (hemoglobin 7 g/dl, ferritin 12 μg/dl, total iron binding capacity (TIBC) 450 μg/dl, and microcytic hypochromic red blood cell (RBCs) on peripheral blood smear), WBC 7500 cells/dl (neutrophils 74%, lymphocytes 23%, monocytes 1%, and eosinophils 2%), and platelet counts of 2.67 lakh/dl. Coagulation profile, liver function test, renal function test, and urine microscopy were normal. Chest X-ray [Figure 1] showed bilateral reticulonodular opacity in mid and lower zones. Sputum acid fast bacilli (AFB) smear was negative. High-resolution computed tomography (HRCT) chest [Figure 2] revealed bilateral ground glass opacity with lower lobe predominance. Immune markers − antinuclear antibodies, rheumatoid factor, antidouble standard deoxyribonucleic acid (DNA), antineutrophil cytoplasmic antibodies, antiglomerular basement membrane antibodies, and antiphospholipid antibodies were negative. Spirometry showed mild restriction and diffusion capacity of the lung for carbon monoxide (DLCO) value was increased. Fiber-optic bronchoscopy was performed. Bronchoalveolar lavage revealed hemosiderin-laden macrophages, and transbronchial lung biopsy from right lower lobe showed alveolar tissue with numerous hemosiderin-laden macrophages in air spaces, which was consistent with pulmonary hemosiderosis. For further confirmation, video-assisted thoracosopic surgery guided lung biopsy was taken from right lower lobe, which on histopathological examination [Figure 3] revealed alveolar hemorrhages and numerous hemosiderin-laden macrophages with the absence of vasculitis, granuloma, and inflammation which was diagnostic of IPH. The patient was started on oral glucocorticoid (prednisolone) at 0.75 mg/kg body weight/day and was continued till clinico-radiological [Figure 4] resolution, which took 2 months. The dose was, subsequently, tapered at the rate of 5 mg every 2 weeks and then stopped. Patient is on regular follow-up till date and has been asymptomatic thereafter.
|Figure 1: Chest X-ray showing bilateral reticulonodular opacity in middle and lower zone|
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|Figure 3: Medium power view showing: (A) Bronchovascular bundle with no vasculitis or bronchial inflammation or granuloma. (B) Hemorrhages and hemosiderin-laden macrophages in alveoli. High magnification showing: (C) Hemosiderin-laden macrophages. No evidence of interstitial inflammation. (D) Small vessels with no evidence of vasculitis|
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|Figure 4: HRCT chest showing resolution of ground glass opacity performed after completion of treatment|
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| Discussion|| |
IPH is a rare disease of unknown etiology. Children before 10 years of age account for 80% of cases of IPH while the remaining cases are found in young adults of <30 years. The age of the patient reported here is 27 years. Hemoptysis is a common presenting complaint for tuberculosis in endemic country such as India. In this case too, the patient was treated for tuberculosis before coming to us. Thus, lack of awareness of the disease and misdiagnosis as tuberculosis further delayed the diagnosis of this rare entity.
Blood investigation, chest X-ray, computed tomography chest, spirometry, bronchoscopy, and lung biopsy are helpful in establishing the diagnosis. The approach to evaluation of DAH includes proper history taking with emphasis on drug history and any history suggestive of vasculitis, immunological disorders, and connective tissue diseases, physical examination, and laboratory tests to rule out coagulopathies and asymptomatic immunological disorders and connective tissue diseases. Chest radiograph typically shows diffuse, bilateral opacity predominantly in the lower zone. These opacities have ground glass characteristics on HRCT. Lung function test shows restriction on spirometry, and DLCO is often increased in acute cases due to increased uptake of carbon monoxide by the red blood cells in the airway while it may be low or normal in chronic cases. In this case, mild restriction with increased DLCO was found.
Cytology of sputum or bronchoalveolar lavage reveals hemosiderin-laden macrophages. Lung biopsy is gold standard for diagnosis. It rules out vasculitis, granulomatous, immunological, and inflammatory cause of DAH.
No guidelines have been formulated for the treatment of IPH. Immunosuppressant splenectomy and lung transplantation have been tried for its treatment. Corticosteroids among immunosuppressant have shown promising results in various trials. Prednisolone is started at a dose of 1 mg/kg/day for a couple of months; the new alveolar infiltrates tend to resolve by then and is subsequently tapered if no symptoms occur.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Lynch JP, Fishbein MC, Wang T. Alveolar hemorrhage syndromes. In: Michael AG, editor. Fishman’s Pulmonary Diseases and Disorders. 5th ed. New York: McGraw-Hill Education; 2015. p. 1001-13.
Milman N, Pedersen FM. Idiopathic pulmonary hemosiderosis. Epidemiology, pathogenic aspects and diagnosis. Respir Med 1998;92:902-7.
Gencer M, Ceylan E, Bitiren M, Koc A. Two sisters with idiopathic pulmonary hemosiderosis. Can Respir J 2007;14:490-3.
Akyar S, Ozbek SS. Computed tomography findings in idiopathic pulmonary hemosiderosis. Respiration 1993;60:63-4.
Ewan PW, Jones HA, Rhodes CG, Hughes JM. Detection of intrapulmonary hemorrhage with carbon monoxide uptake. Application in Goodpasture’s syndrome. N Engl J Med 1976;295:1391-6.
Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J 2004;24:162-70.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]