Journal of Postgraduate Medicine
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CASE REPORT
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Year : 2013  |  Volume : 59  |  Issue : 2  |  Page : 145-148  

Aspergilloma coexisting with idiopathic pulmonary fibrosis: A rare occurrence

N Kumar, M Mishra, A Singhal, J Kaur, V Tripathi 
 Department of Respiratory Medicine, Chhatrapati Shivaji Subharti Hospital, Subharti Medical College, Meerut, Uttar Pradesh, India

Correspondence Address:
M Mishra
Department of Respiratory Medicine, Chhatrapati Shivaji Subharti Hospital, Subharti Medical College, Meerut, Uttar Pradesh
India

Abstract

Fungal ball (mycetoma/aspergilloma) is a saprophytic fungal infection that colonizes pre-existing lung cavities. Reported literature suggests its development in cystic lesions/cavitation associated with tuberculosis, sarcoidosis, bronchiectasis, lung abscess, and cavitating neoplasm to name a few. Coexistence of aspergilloma with idiopathic pulmonary fibrosis (IPF) has not been reported in literature so far, to the best of our knowledge. We hereby report the case of a 55-year-old female with IPF having fungal ball.



How to cite this article:
Kumar N, Mishra M, Singhal A, Kaur J, Tripathi V. Aspergilloma coexisting with idiopathic pulmonary fibrosis: A rare occurrence.J Postgrad Med 2013;59:145-148


How to cite this URL:
Kumar N, Mishra M, Singhal A, Kaur J, Tripathi V. Aspergilloma coexisting with idiopathic pulmonary fibrosis: A rare occurrence. J Postgrad Med [serial online] 2013 [cited 2019 Oct 15 ];59:145-148
Available from: http://www.jpgmonline.com/text.asp?2013/59/2/145/113841


Full Text

 Introduction



Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown cause with a natural history of gradual and progressive breathlessness. Basal, peripheral reticular opacities, and associated traction bronchiectasis and honeycombing are typical high-resolution computed tomography (HRCT) signs of IPF in most cases. [1],[2] These findings, combined with the clinical profile, are sufficient to establish a confident diagnosis of IPF, even in the absence of a surgical lung biopsy. [2]

Aspergilloma is essentially a fungus ball that develops as a saprophytic fungal infection in the lung. The most common predisposing factor for the formation of an aspergilloma appears to be the presence of a pre-existing lung cavity, [3] as seen in tuberculosis, sarcoidosis, bronchiectasis, lung abscess, and cavitating carcinoma, among others. Development of aspergilloma has been reported most commonly in association with post-tubercular cavities, the reported frequency being in the range of 11-17% [4] to 4-20%. [5]

Both IPF and aspergilloma are commonly diagnosed diseases in India. However, a thorough MEDLINE search remains mostly silent on any available literature describing the coexistence of aspergilloma with IPF. The rarity of this association prompted us to report the case.

 Case Report



A 55-year-old female presented to our hospital in October 2010 with a history of dry cough and progressive shortness of breath of 6 months duration. There was no history of chest pain, fever, hemoptysis, dysphagia, joint pains, myalgia, loss of appetite or weight, swelling over feet/face, skin/eye lesions, or any urinary complaints. There was no history of exposure to pets, molds, asbestos, or silica. There was no past history of pulmonary tuberculosis. The patient was a never-smoker and there were no other comorbid medical illnesses. At that time, her physical examination revealed clubbing (grade 2) and bilateral basal end-inspiratory velcro crackles. Routine blood investigations were within normal limits. Chest X-ray postero-anterior (PA) view showed bilateral reticular pattern in lower lung fields. HRCT-thorax was suggestive of bilateral inter- and intra-lobular septal thickening, subpleural honeycombing, areas of tractional bronchiectasis mainly in lower lobes and reduced lung volume. It also showed patchy ground-glass opacities with intervening normal lung, giving a variegated appearance [Figure 1]. Her echocardiography (ECHO) revealed a left-ventricular ejection fraction of 67% and mild pulmonary arterial hypertension (PAH). Pulmonary function tests (PFT) showed moderate restriction. Diffusion capacity of the lung for carbon monoxide was reduced and 6-minute walk test (6MWT) revealed a walk-distance of 100 m. Arterial blood gas analysis (ABG) showed partial pressure of carbon dioxide (PaCO 2 )-45 mmHg, partial pressure of oxygen (PaO 2 )-90 mmHg, and arterial oxygen saturation (SPO 2 )-90% on room air. Rheumatoid factor, anti-nuclear antibodies, anti-dsDNA, and anti-neutrophil cytoplasmic antibodies were negative. Serum angiotensin converting enzyme level was found to be normal. Fiberoptic bronchoscopy was performed and bronchoalveolar lavage fluid was inconclusive. Transbronchial lung biopsy report was suggestive of a usual interstitial pneumonia (UIP) pattern. Based on the clinical presentation, HRCT-thorax findings, histopathological report, and the exclusion of other possible causes, she was diagnosed as a case of IPF. She was treated with oral corticosteroids and other supportive management. The patient was subsequently lost to follow-up after 6 months.{Figure 1}

She again presented to our hospital in August 2012 with worsened cough and shortness of breath. Her physical examination revealed cyanosis, tachypnoea, bilateral pedal edema, raised jugular venous pressure, and grade 3 clubbing. Extensive bibasilar end-inspiratory velcro crackles were heard all over her chest. The patient was managed in a respiratory intensive care unit (ICU). Her chest X-ray PA view showed an enlarged cardiac silhouette, reticulo-nodular pattern of interstitial prominence over both lung fields, and haziness in lower zone of right lung. HRCT thorax showed extensive bilateral inter- and intra-lobular septal thickening, sub-pleural honeycombing, areas of profuse tractional bronchiectasis in both lungs, and fungal ball in right lower lobe with air crescent sign [Figure 2]a and b. Her ECHO revealed normal left ventricular size and function, dilated right atrium and right ventricle, mild tricuspid regurgitation, and severe PAH (PASP 75 mmHg). PFT showed mixed pattern with predominant restriction. ABG analysis showed PCO 2 , 51 mmHg; PO 2 , 70 mmHg; and SPO 2 , 70%, on room air. 6-MWT could not be performed by the patient. Bronchoscopy and bronchoalveolar lavage could not be done because of her low general condition and negative consent by the attendants. Her sputum stains and cultures were negative for Gram stained organisms, acid fast bacilli and fungal elements. Serum IgE level was normal. During her ICU stay, she was treated with supplemental oxygen, nebulized bronchodilator and anticholinergic, intravenous empirical antibiotics, bronchodilators, diuretics, corticosteroids, and oral sildenafil. Systemic antifungal therapy in the form of oral itraconazole (200 mg, twice daily) was added in view of the CT finding of aspergilloma. A surgical opinion was sought for aspergilloma, but no active surgical intervention was advised because of severe underlying pulmonary dysfunction. After 5 days of ICU stay, the patient was maintaining SPO 2 of 88-90% on 1-2 l/min oxygen flow via nasal prongs and was able to walk a few yards with support. Thereafter, she was shifted to the high dependency unit. The patient was discharged on request after 3 days on oral pirfenidone, diuretics, sildenafil, methyl prednisolone, itraconazole, long-term oxygen therapy, nebulized bronchodilator, and anticholinergic. The patient visited the outpatient after 1 week in a slightly better general condition.{Figure 2}

 Discussion



IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathological and/or radiological pattern of UIP. [6] The diagnosis of IPF essentially requires the following: (a) Exclusion of other known causes of interstitial lung disease (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity); (b) The presence of a UIP pattern on HRCT in patients not subjected to surgical lung biopsy; (c) Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. In the present case, the diagnosis of IPF was made on the basis of guidelines laid down in the official statement of the American Thoracic Society. [6]

Pulmonary aspergillosis can manifest in different forms depending upon the underlying lung architecture and immune status of the host. Thus, we may have a simple colonization of the fungus in the respiratory tract; hypersensitivity reactions such as allergic bronchopulmonary aspergillosis, hypersensitivity pneumonitis, or bronchocentric granulomatosis; saprophytic growth of the fungus (aspergilloma); or invasive pulmonary aspergillosis. [4],[7],[8]

Saprophytic colonization of a parenchymal lung cavity by Aspergillus is referred to as aspergilloma, mycetoma, or a fungus ball. It usually represents a non-invasive form of aspergillosis. [9] A fungus ball consists of both dead and living mycelial elements, fibrin, mucus, amorphous debris, inflammatory cells, and degenerating blood and epithelial elements. Most aspergillomas are caused by A. fumigatus, but some are caused by A. niger (especially in diabetics). Chronic pulmonary aspergillosis is defined in a non-immunocompromised patient in the presence of 1) at least one pulmonary cavity on thoracic imaging; 2) positive anti-aspergillus immunoglobulin G antibodies (precipitins) in blood, cultures, or biopsy implicating Aspergillus species; and 3) either symptoms (usually weight loss, fatigue, cough, hemoptysis, and breathlessness) for >3 months, with slowly progressive destruction of the lung with or without one or more fungal ball in a cavity (chronic cavitary pulmonary aspergillosis or chronic fibrosing pulmonary aspergillosis), or a fungal ball in a cavity without evidence of progression and few or no symptoms (simple aspergillomas). [10]

The most common disorders with which occurrence of aspergilloma has been described are tuberculosis, asbestosis, sarcoidosis, lung abscess, cavitary neoplasia, [11] bronchiectasis, emphysema, pulmonary infarction, [12] histoplasmosis, ankylosing spondylitis, bronchial cysts, [13] hydatid cyst, [14] Wegener's granulomatosis, [13],[15],[16] berryliosis, [17] silicosis, [18] asbestosis, [19] and allergic bronchopulmonary aspergillosis. [20] Few isolated case reports also exist linking the existence of aspergilloma in hypersensitivity pneumonitis, [8],[21] chronic interstitial pneumonia, [22] and interstitial lung disease. [9] However, its occurrence with IPF has not been mentioned in previously reported literature to the best of our knowledge and hence this case is being reported.

The diagnosis of aspergilloma usually requires clinical, radiological, and serological evidence. Clinically, the patient may be asymptomatic or present with hemoptysis. Radiological diagnosis is made upon visualizing a well-defined heterogeneous density within a pre-formed cyst cavity (usually solitary in an upper lobe), often separated from the cyst wall by an air crescent. CT usually reveals globules of gas within the hyphal ball, which may be loose or attached to the cavity wall by granulation tissue. With effort, the fungus ball can often be shown to be mobile within the cavity when the ball does not fill the entire cavity. [23] In our patient, the CT thorax showed fungal ball in the right lower lobe with air-crescent sign [Figure 2]a, which moved when imaging was done in the prone position [Figure 2]b. Although sputum cultures are positive for Aspergillus in more than half of patients with aspergilloma, it is not a sensitive and specific diagnostic marker. In the present case, it was negative. Precipitating antibodies to Aspergillus antigens are present in the sera of >95% of patients with aspergilloma. Aspergilloma has no effective treatment other than surgery, which is reserved for the severely symptomatic patient, usually with massive hemoptysis. [24] Surgical intervention was not done in our case as the patient's underlying lung condition was severely compromised and there was no hemoptysis.

It is well-known that aspergilloma occurs in pre-formed cavitary lesions in various disorders, whereas IPF is usually not associated with cavitation. So what could possibly explain their coexistence? The answer may lie in the extensive honeycombing and cystic lesions of IPF together with its debilitating, chronic nature, as well as the immunosuppressive effect of long term steroid/other immunosuppressive therapy that could be partially responsible for the development of aspergilloma in IPF. However, the exact mechanism needs further evaluation.

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