|Year : 2006 | Volume
| Issue : 2 | Page : 90-92
An unusual cause of hilar lymphadenopathy
Ritesh Agarwal, Chandana Reddy, D Gupta
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh., India
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh.
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Allergic bronchopulmonary aspergillosis is an uncommon condition characterized by hypersensitivity to aspergillus antigen. It commonly presents as refractory asthma, fleeting pulmonary opacities and bronchiectasis. The authors describe an unusual presentation of allergic bronchopulmonary aspergillosis, when it was mistaken as bronchogenic carcinoma and review the current literature on the diagnosis and management of this rare condition.
Keywords: Hilar lymphadenopathy, Allergic bronchopulmonary aspergillosis
|How to cite this article:|
Agarwal R, Reddy C, Gupta D. An unusual cause of hilar lymphadenopathy. Lung India 2006;23:90-2
| Introduction|| |
Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder characterized by hypersensitivity to fungi most commonly Aspergillus fumigatus, a fungus which colonizes the bronchial mucus of patients with asthma and cystic fibrosis. It occurs in 1-2% of patients with bronchial asthma and 2-15% of patients with cystic fibrosis  . The immunological substrate of this hypersensitivity includes a T H 2 CD4+ lymphocyte response, IgE mediated (type 1) and IgG mediated (type 3) hypersensitivity. Patients often present with unremitting asthma and other systemic complaints like fever, anorexia, weight loss and hemoptysis. Common radiological manifestations include fleeting pulmonary infiltrates, bronchoceles (mucus filled bronchi), consolidation, nodules and bronchiectasis. The diagnosis is often missed and infact many patients are misdiagnosed as pulmonary tuberculosis, because of the upper lobe opacities and bronchiectasis. We describe an uncommon presentation of ABPA, wherein the patient was initially diagnosed as bronchogenic carcinoma and even underwent fiberoptic bronchoscopy in the diagnostic evaluation. Proper clinical suspicion and serology confirmed the diagnosis.
| Case Report|| |
A 60 year-old female, presented with history of cough with minimal mucoid expectoration and exertional breathlessness of six years duration. She also had history of winter exacerbations associated with wheezing. She was a post-menopausal lady, non smoker, with history of exposure of biomass fuel. For the last six months, she complained of increasing dyspnea, dull aching left sided chest pain, anorexia and weight loss of 5kg. There was no history of fever or hemoptysis. On examination she had pallor. Rest of the physical examination was unremarkable. A general practitioner had investigated her and a chest radiograph was obtained (Figure 1). The chest radiography showed left hilar prominence, and nonhomogenous opacities in left middle and lower zone. A flexible fiberoptic bronchoscopy examination performed at another center, suspecting a bronchogenic carcinoma, was normal. She was referred to our center, where a review of chest radiograph showed left hilar prominence and pulmonary opacities confirming to the morphology of glove-in-finger suggesting the presence of bronchiectasis and mucus filled bronchi. With the history suggesting bronchial asthma, and the presence of bronchiectasis, a diagnosis of allergic bronchopulmonary aspergillosis (ABPA) was made. High-resolution computed tomography (HRCT) confirmed the presence of bronchiectasis and also shows numerous bronchoceles (mucus filled bronchi) which appear as hilar lymphadenopathy (Figure 2). A skin test for Aspergillus fumigatus was positive; her total IgE count was 11098 IU/mL, IgE specific to Aspergillus fumigatus 42 U/L, absolute eosinophil count was 2000/mL, and there were presence of apergillus precipitins. The final diagnosis made was allergic bronchopulmonary aspergillosis.
| Discussion|| |
Allergic bronchopulmonary aspergillosis is one of the common causes of eosinophilic lung disease and probably the most common cause of eosinophilic pneumonia in the developed countries  . Patients may present with recurrent asthma exacerbations, expectoration of dark brown mucus plugs, hemoptysis of systemic features such as fever, anorexia, malaise and weight-loss. There is no specific physical finding associated with ABPA. The radiological findings include "gloved-finger", "tooth-paste" shadows, airfluid levels from dilated bronchi and tramline shadows from edematous bronchial walls  . HRCT characteristically shows central bronchiectasis, mucusfilled bronchi (also called bronchoceles), consolidation and centrilobular nodules  . Bronchoceles are usually proximal, often inseparable from the hilum and may simulate hilar lymphadenopathy and is probably the cause of left hilar mass in our patient  . The Rosenberg criteria are most widely used for diagnosis (Box 1)  . Patients may also present with less than the full complement of diagnostic criteria. Greenberger  proposed the use of clinical designations ABPA-S (seropositive) and ABPA-CB (central bronchiectasis) and emphasized that bronchiectasis is not an essential criteria.
The natural history of ABPA is characterized by clinical, immunological and radiologic episodes of remission and relapse. Five stages are commonly described which do not necessarily progress in a sequential fashion. Stage I is the acute stage in which patients fulfil the Rosenberg criteria; with treatment the abnormalities subside, and if resolution persists for six months, the patient is considered to be in stage II (remission). Traditionally, patient is said to be in remission, if the IgE levels decline by atleast 35% before they plateau. Stage II may persist indefinitely or the patient may have recurrence, classically defined as doubling of total IgE levels-stage III (relapse). Stage IV (glucorticoid-dependent asthma) represents those patients with or without infiltrates on chest radiograph, who continue to have persistently raised IgE levels despite ongoing glucorticoid use and withdrawal of the drug results in exacerbation of the disease. Stage V includes patients with widespread fibrosis and bronchiectasis.
Glucocorticoids are the cornerstone of therapy. They have to be given at a dose of 0.5mg/kg/day oral prednisolone for 1-2 weeks, then on alternate days for 6-8 weeks and tapered at 5-10mg every 2 weeks and discontinued. The aim is to decrease the inflammation and attenuate the fungal antigen burden. Patients are followed up with serum IgE levels repeated every 6-8 weeks until remission and then annually with serial chest radiographs, pulmonary function tests and IgE levels. Importantly the treatment is not aimed at normalizing the IgE levels, but only to decrease it by 35%. The role of itraconazole is still not clear; however most authorities would give it as corticosteroid-sparing agent at doses of 200mg twice a day for 16 weeks and then once a day for 16 weeks. It has been shown to decrease the corticosteroid requirement and airway inflammation, and can be given as a corticosteroid-sparing agent. ,
Box 1: Diagnostic criteria for ABPA (Rosenberg criteria)
- Asthma or cystic fibrosis
- Peripheral blood eosinophilia
- Immediate cutaneous reactivity to aspergillus antigen
- Precipitins against aspergillus antigen
- Elevated total serum IgE (> 1000 IU/mL)
- Fleeting pulmonary infiltrates
- Central bronchiectasis
- Elevated specific IgE and IgG to aspergillus antigen
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