|Year : 2019 | Volume
| Issue : 3 | Page : 170-173
Idiopathic pulmonary hemosiderosis: A differential diagnosis of pulmonary tuberculosis in a young child
Shelley Shamim, Suptish Biswas, Suman Mandal
Department of Pulmonary Medicine, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India
|Date of Submission||19-Apr-2019|
|Date of Decision||02-Jun-2019|
|Date of Acceptance||08-Jun-2019|
|Date of Web Publication||19-Aug-2019|
Dr. Suptish Biswas
Sarada Sarani Road, PO Hridaypur, PS Barasat, Kolkata - 700 127, West Bengal
Source of Support: None, Conflict of Interest: None
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease diagnosed primarily in children and young adults, characterized by recurrent diffuse alveolar hemorrhage which leads to the deposition of hemosiderin-laden macrophages in the lung. It is a diagnosis of exclusion and requires a high level of clinical suspicion. It may remain undiagnosed for years, hence often mistreated. Here, we describe such a case of IPH in a 15-year-old male who was initially mistreated twice as clinically diagnosed pulmonary tuberculosis first with Category 1 and then Category 2 antitubercular drugs. He had a history of two episodes of hemoptysis, recurrent episodes of paleness, persisting weakness, and shortness of breath. He also had bilateral reticulonodular opacities on chest skiagram. His history raised the suspicion, the presence of hemosiderin-laden macrophages in bronchoalveolar lavage, and transbronchial lung biopsy strongly suggested the diagnosis. However, the final diagnosis came out after ruling out all the probable causes.
Keywords: Hemosiderin-laden macrophages, idiopathic pulmonary hemosiderosis, transbronchial lung biopsy
|How to cite this article:|
Shamim S, Biswas S, Mandal S. Idiopathic pulmonary hemosiderosis: A differential diagnosis of pulmonary tuberculosis in a young child. Indian J Med Spec 2019;10:170-3
|How to cite this URL:|
Shamim S, Biswas S, Mandal S. Idiopathic pulmonary hemosiderosis: A differential diagnosis of pulmonary tuberculosis in a young child. Indian J Med Spec [serial online] 2019 [cited 2022 Jan 18];10:170-3. Available from: http://www.ijms.in/text.asp?2019/10/3/170/264525
| Introduction|| |
Idiopathic pulmonary hemosiderosis (IPH) is a very rare entity in the broad spectrum of diffuse alveolar hemorrhage, with a reported incidence of 0.24–1.23 cases per million in selected populations, and is found primarily in children and young adults, characterized by recurrent hemoptysis, dyspnea, anemia, and alveolar opacities on chest skiagram. Recurrent alveolar hemorrhage results in deposition of hemosiderin-laden macrophages in the lung. Here, we are presenting a case of IPH in a 15-year-old male, mistreated initially as clinically diagnosed pulmonary tuberculosis.
| Case Report|| |
A 15-year-old male presented with low-grade intermittent fever with dry cough for 10 days and shortness of breath of the modified Medical Research Council (mMRC) Grade 1. He had a history of one episode of mild hemoptysis 5 days back, followed by which he progressively became pale over a few hours. It was associated with generalized severe weakness and increased breathlessness. He received 2 units of packed red blood cells transfusion in a local medical facility and was referred to our institution. He had a history of similar type of episode without hemoptysis 3 years back and another episode with mild hemoptysis 5 years back. He was conservatively managed at local peripheral health care and recovered from paleness within 2 to 3 days during the past episodes, without any blood transfusion. Since the first episode, 5 years back, he was experiencing mMRC Grade 1 shortness of breath. After the first episode, 5 years back, he was treated with Category 1 antitubercular drugs (ATDs) as a case of clinically diagnosed pulmonary tuberculosis. After the second episode, he was treated with category two ATDs as a case of treatment failure pulmonary tuberculosis. He had no history of night sweat, weight loss, skin rash, or joint pain.
On examination, the patient had mild pallor, blood pressure – 126/78 mmHg, pulse rate – 86/min, respiratory rate – 20/min, temperature – 97°F, SpO2– 98% in room air, and body mass index – 16.2. There was no palpable lymphadenopathy. Respiratory examination revealed bilaterally few discrete crepitations in the mid to lower zone. Cardiac, abdominal, and nervous system examinations were within the normal limits.
Initial laboratory investigations after we received the patient in our institution showed hemoglobin (Hb)% – 11.3 g/dl, total leukocyte count – 7400/mm 3 (N: 59, L: 26, M: 02, E: 13, B: 00), erythrocyte sedimentation rate – 52 mm in 1 h, and reticulocyte index 4.5. Renal function, electrolytes, and coagulation profile were normal with only mild elevated aspartate transaminase (serum glutamic oxaloacetic transaminase: 69 U/L) and alanine transaminase (serum glutamic pyruvic transaminase: 96 U/L) in liver function test. Sputum for acid-fast bacilli, Gram-stain and culture, and cartridge-based nucleic acid amplification test (CBNAAT); urine for routine examination and culture; stool for occult blood test and routine examination, malarial parasite antigen detection test, and Typhidot M; hepatitis B virus surface antigen; anti-hepatitis C virus; and HIV antibodies testing were negative. Serum iron was 91 mcg/dl, total iron-binding capacity was 487 mcg/dl, transferrin saturation was 18.70%, and ferritin was 276.36 ng/ml. C-reactive protein was 6.6 mg/L. The Mantoux test report was available. It was done previously after the first episode and it was positive with 15 mm × 16 mm induration.
Cardiac evaluation was within the normal limits. Pulmonary function test (PFT) with diffusion capacity (diffusion capacity of the lung for carbon monoxide [DLCO]) was done. The results were suggestive of normal pulmonary function with reduced diffusion capacity (DLCO: 52% of predicted).
Chest radiograph showed bilateral reticulonodular opacities predominantly involving the mid and lower zones [Figure 1]. Chest computed tomography (CT) scan showed bilateral diffuse ground-glass opacity with centrilobular nodules and mildly enlarged mediastinal lymph nodes [Figure 2].
|Figure 1: Chest X-ray showing bilateral reticulonodular opacity predominantly involving mid and lower zones|
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|Figure 2: Chest computed tomography scan showed bilateral diffuse ground-glass opacity with centrilobular nodules and mildly enlarged mediastinal lymph nodes|
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Bronchoalveolar lavage (BAL) showed faint brownish fluid, which on cytological examination showed plenty of hemosiderin-laden macrophages [Figure 3]. Gram-stain, culture, and CBNAAT from the BAL fluid sample were negative.
|Figure 3: Bronchoalveolar lavage fluid examination showing plenty of hemosiderin-laden macrophages (blue)|
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Transbronchial lung biopsy was performed. Histopathological examination of biopsy sample showed the lung interstitium, and the alveoli are infiltrated by a large number of macrophages containing brown pigment, which were confirmed as hemosiderin on Perl's stain. There was no evidence of vasculitis, epithelioid granuloma, or tuberculosis or malignancy [Figure 4] and [Figure 5].
|Figure 4: The intersitium and alveoli are infiltrated by large number of macrophages containing brown pigment (H and E)|
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|Figure 5: Brown pigment contained in macrophages, stained blue, and iron (hemosiderin) (Prussian blue stain)|
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Workup to rule out immunological causes was done, which included proteinase 3 anti-neutrophil cytoplasmic antibody (ANCA), myeloperoxidase-ANCA, antinuclear antibody profile, antiphospholipid antibody (IgM and IgG), anti-glomerular basement membrane (GBM) antibody, antihuman tissue transglutaminase antibody, anti-endomysial antibody (IgA), and C3 and C4. All these investigations were tested as negative.
The case was stamped as IPH. The treatment was started with oral prednisolone 35 mg daily (1 mg/kg of body weight [BW]) and was continued for 1 month. The patient showed clinical improvement following this treatment initiation. After 1 month of therapy, the dose of oral prednisolone was gradually tapered off by 2.5 mg/week. The patient was discharged with oral prednisolone (25 mg/day). As he maintained his improved clinical status in spite of gradual dose reduction, oral prednisolone gradually tapered off to 7.5 mg/day. The patient continued to be on oral prednisolone (7.5 mg/kg) and has shown no sign of clinical deterioration or any evidence of recurrence at his 12th month follow-up.
| Discussion|| |
Many underlying entities can lead to diffuse alveolar hemorrhage, e.g., systemic vasculitis (granulomatosis with polyangiitis, Henoch–Schönlein purpura, and microscopic polyangiitis), rheumatic diseases (anti-GBM disease, isolated pulmonary capillaritis [ANCA positive], antiphospholipid antibody syndrome, and systemic lupus erythematosus), infections, drugs, coagulopathies, and mitral valve disease. If no underlying cause is apparent, the entity is referred to as IPH.,
High-resolution CT scan demonstrates unilateral or bilateral ground-glass opacities with occasional centrilobular nodules. The presence of parenchymal fibrosis may also be evident in untreated patients, experiencing recurrent episodes.
PFT usually demonstrates restrictive pattern. After an acute episode, DLCO rises inappropriately because of a large number of erythrocytes that have spilled into the alveoli avidly bind carbon monoxide. Degradation of Hb reduces DLCO to prehemorrhage state. If there is parenchymal fibrosis due to untreated repeated episodes, DLCO may remain chronically low.
Absence of any evidence of capillaritis is a key feature of IPH.,
Timely diagnosis, introduction of systemic glucocorticoids with gradual dose tapering and close monitoring is the basis of treatment. Glucocorticoids also limit the progression of pulmonary fibrosis. Oral prednisolone of 0.5–0.75 mg/kg of BW/day is usually used for induction of treatment. It is continued until active intra-alveolar bleeding stops, and chest skiagram shows regression of newly acquired opacities. Treatment should be tapered and discontinued after 18–24 months if there is no recurrence. However, the duration of chronic therapy must be individualized. Hydroxychloroquine and azathioprine have shown some beneficial effects in some corticosteroid refractory cases.
Our patient, here, had three episodes over 5-year duration. Despite not having typical features, during the first two episodes, he was mistreated with ATDs as a case of clinically diagnosed pulmonary tuberculosis, at peripheral health care. Lack of clinical awareness about diffuse alveolar hemorrhage and the trend of clinically diagnosing pulmonary tuberculosis by chest skiagram and few supporting clinical features, without thorough evaluation, were the main reasons behind the delayed proper diagnosis of this case of IPH and unnecessary exposure of the patient to ATDs and the risks of their adverse effects. The knowledge of India being a tuberculosis-endemic country also played a crucial role here.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]