Pulmonary Haemosiderosis Secondary to Mitral Valve Stenosis-Known Association of Uncommon Entity: A Case Report
Correspondence Address :
Dr. Chandrasekhar Patil,
Assistant Professor, Department of Radiology, Mallareddy Medical College for Women, Suraram, Hyderabad-500055, Telangana, India.
E-mail: drchandruhbli@gmail.com
Pulmonary haemosiderosis is a rare lung disease characterised by haemosiderin deposition in the lungs due to recurrent intra-alveolar bleeding. It can be idiopathic or secondary due to mitral stenosis. Affected patients will have symptoms of cough, haemoptysis, and shortness of breath. Radiologically, it is characterised by widespread tiny alveolar nodular opacities with or without septal thickening. Therefore, in the case of mitral valve stenosis, this imaging pattern on High-resolution Computed Tomography (HRCT) should prompt suspicion of secondary pulmonary haemosiderosis. Here, the authors present the case of a 27-year-old male patient with mitral stenosis who presented with progressive shortness of breath, mild atypical chest pain for two months, and a cough with occasional haemoptysis in the last 10 days before the hospital visit, along with typical radiological findings of widespread tiny ground glass alveolar nodules on HRCT. The radiological knowledge of this rare entity is very important for confidently making the diagnosis in the proper clinical context, to avoid unnecessary investigations and treatment.
Haemoptysis, High-resolution computed tomography, Nodular opacities, Septal thickening
A 27-year-old male patient with a history of Chronic Rheumatic Heart Disease (CRHD) and severe mitral stenosis presented to the hospital with complaints of progressive shortness of breath, mild atypical chest pain for the past two months, and occasional cough with haemoptysis in the 10 days before the hospital visit. There was no history of cold, fever, weight loss, loss of consciousness, or palpitations. On clinical examination, the patient was conscious and coherent with the following vital signs: blood pressure of 110/70 mmHg, pulse 106 bpm, respiratory rate of 38 cycles/min, and peripheral oxygen saturation (SpO2) of 80%. The rest of the physical examination was unremarkable. However, auscultation of heart sounds revealed a loud S1, an opening snap, a diastolic rumble, and fine crackles in bilateral lung bases. A 2D Echocardiography (Echo) was performed due to abnormal auscultatory findings, revealing significant Mitral Stenosis (MS) with a valve area of 1 sqcm, thickened and calcified valve tips, doming of the anterior mitral leaflet with a fixed posterior mitral leaflet, a thickened annulus of the aortic valve (19 mm), and a dilated left atrium measuring 53 mm. The complete blood picture of the patient revealed low haemoglobin of 8.0 gm%, with a normal total leucocyte count of 5400 cells/cumm and platelet count of 3.88 lacs/cumm.
Based on the clinical examination and 2D ECHO cardiography, the patient was diagnosed with moderate pulmonary arterial hypertension {Right Ventricular Systolic Pressure (RVSP)-60 mHg} in addition to severe mitral stenosis, moderate Aortic Regurgitation (AR), and Tricuspid Regurgitation (TR). A chest X-ray was performed on admission, showing mild cardiomegaly. The patient was then advised to undergo HRCT of the chest to look for lung abnormalities. The HRCT chest revealed multiple tiny innumerable centrilobular nodules involving bilateral lower lobes. Segmental consolidation with surrounding glassing was noted involving the right middle lobe, and multifocal patchy ground glassing was noted in the anteromedial segment of the left lower lobe and segments of the right lower lobe [Table/Fig-1a-f]. Given the clinical history, a radiological diagnosis of pulmonary haemosiderosis due to chronic recurrent alveolar haemorrhage secondary to mitral stenosis was made. The patient was advised to undergo bronchoalveolar lavage; however, the patient refused the procedure, which is the ideal investigation for confirmation.
The patient was started on methylprednisolone 16 mg BD, which was gradually tapered, montelukast levocetirizine OD, levocloperastine 10 mL TID, ferrous fumarate, and folic acid BD. After the treatment commenced, the patient’s symptoms improved during a follow-up of 6 to 7 weeks. Later, the patient was referred to the cardiology unit, where mitral valve replacement was advised.
Pulmonary haemosiderosis is a rare disease that occurs due to the deposition of haemosiderin-laden macrophages in the lungs as a result of repeated alveolar haemorrhages. Pulmonary haemosiderosis is divided into two main types: 1) idiopathic; and 2) secondary due to mitral valve stenosis, collagen vascular diseases, and coagulation disorders.
The pulmonary system responds to haemorrhage via activation of alveolar macrophages. These specialised macrophages phagocytose erythrocytes at a rate six times slower than systemic macrophages (1). This reduced ability to metabolise the haemorrhaged blood ultimately leads to a build-up of haemosiderin (1). Idiopathic Pulmonary Haemosiderosis (IPH) is a rare condition in which the patient suffers from recurrent episodes of diffuse alveolar haemorrhage of unknown aetiology (2),(3).
The toxic effect of iron is exhibited by the production of highly reactive hydroxyl radicals (Haber-Weiss and Fenton reactions), which in turn, leads to the peroxidation of the lipid layer, carbohydrate and protein degeneration, and consequent fibrogenesis. Inside the pulmonary macrophages, the enzyme heme oxygenase removes the iron from haemoglobin, however, the volume of alveolar macrophages to metabolise the iron is restricted, and the existence of free iron in the alveoli can lead to local injury and fibrosis.
The clinical manifestations of haemosiderosis may include iron deficiency anaemia, repeated episodes of haemoptysis, and diffuse pulmonary infiltrates (4).
Plain radiograph findings are usually non specific and depend on the acuity and severity of alveolar haemorrhage. Radiograph findings may be subtle in pulmonary haemosiderosis or unremarkable (2), however, HRCT of the chest is a more sensitive investigation as it reveals the subtle findings that can be missed on plain radiographs. The imaging features of pulmonary haemosiderosis on HRCT would be ground glassing, consolidation, randomly scattered miliary nodules, or a combination of findings. The crazy paving pattern is also described in pulmonary haemosiderosis, which is due to the deposition of haemosiderin in interlobular septa. In chronic cases, due to repeated haemorrhage, there would be secondary changes of traction bronchiectasis, subpleural honeycombing, bronchiectasis, and even massive pulmonary fibrosis can be seen as long-term complications of pulmonary haemosiderosis (5).
The definitive diagnosis of pulmonary haemosiderosis is usually established by bronchoalveolar lavage and lung biopsy, which demonstrates diffuse alveolar haemorrhage and haemosiderin-laden macrophages in the alveoli and to some extent in the interstitium.
The most common differential diagnosis for pulmonary haemosiderosis with the widespread miliary pattern is tuberculosis and miliary metastasis. In tuberculosis, there will be consolidation, mediastinal lymphadenopathy, and a history of chronic low-grade fever and weight loss in addition to cough with expectoration. Miliary metastasis is usually seen in thyroid and renal carcinomas. In the present case, the tiny military nodules were of ground-glass attenuation, and the close differential for which is hypersensitivity pneumonitis. However, there was no history of exposure to an allergen, and since there is a cough with haemoptysis in the context of severe mitral stenosis, a radiological diagnosis of pulmonary haemosiderosis was made. However, the patient refused to undergo bronchoalveolar lavage, which is the ideal investigation for confirmation. In the present case report, we have referred to a few previous literature cases to support our diagnosis (Table/Fig 2) (6),(7),(8).
The definitive treatment for pulmonary haemosiderosis secondary to heart valve disease includes valve repair and/or valve replacement (5), steroids, and antitussive medications as a supportive agent, while IPH is treated with corticosteroids alone or in combination with other immunosuppressive regimens.
Pulmonary haemosiderosis due to mitral stenosis is a rare disease that can present with multiple miliary nodules in the lung. Therefore, one should have radiological knowledge of these imaging findings to correctly diagnose and avoid misinterpretation, which otherwise can lead to an array of unnecessary investigations and other workups. Here, the authors present an uncommon case with classical imaging findings of widespread miliary ground glass lung nodules, which in the context of severe mitral stenosis, strongly suggests pulmonary haemosiderosis.
DOI: 10.7860/JCDR/2024/68790.18981
Date of Submission: Nov 29, 2023
Date of Peer Review: Dec 16, 2023
Date of Acceptance: Dec 23, 2023
Date of Publishing: Feb 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. NA
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Dec 01, 2023
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ETYMOLOGY: Author Origin
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