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Q1: What is the diagnosis of this respiratory condition?
The diagnosis in this case is allergic bronchopulmonary aspergillosis (ABPA). ABPA occurs in 6%–20% of individuals with asthma and approximately 10% of individuals with cystic fibrosis.1 It is characterised by repeated episodes of exacerbations interspersed with periods of remissions. Diagnostic criteria include (1) asthma, (2) fleeting pulmonary opacities on chest radiographs, (3) positive skin tests for Aspergillusfumigatus, (4) peripheral blood eosinophilia, (5) precipitating antibodies to A fumigatus, (6) raised serum IgE levels, and (7) central bronchiectasis.2 Other minor criteria include expectoration of golden brown sputum plugs, positive sputum culture for aspergillus species and late (Arthus-type) skin reactivity to A fumigatus.
If the disease is not diagnosed early and treated adequately, lung damage continues to progress silently leading to fibrosis in the upper lobes.3 The upper lobe fibrosis of ABPA can mimic pulmonary tuberculosis and patients are often unnecessarily treated with antituberculosis therapy as in our case.
Q2: Which respiratory diseases have coexistent sinus and orbital involvement and what was the cause in this patient?
Allergic and granulomatous diseases commonly involve the lung, sinuses, and the orbit (box 1).4 The diagnosis in this condition was allergic fungal sinusitis with orbital aspergillosis in a patient with allergic bronchopulmonary aspergillosis.
Q3: What is the significance of C-ANCA in chronic lower respiratory tract infections?
Positive C-ANCA is characteristic of Wegener’s granulomatosis. However, in a study done by Ohno et al raised myeloperoxidase ANCA and bactericidal/permeability increasing protein ANCA levels were found in patients with chronic lower respiratory infection.5 Proteinase-3 C-ANCA was also positive in a single case each of the 21 diffuse panbronchiolitis and 16 bronchiectasis patients included in the study. The raised C-ANCA levels found in our patient can thus be explained by the fact that he had underlying chronic respiratory tract infection.
Box 1: Allergic and granulomatous diseases
– Classical Wegener’s granulomatosis.
– Necrotising sarcoid granulomas.
– Behçets disease.
– Midline granulomas.
– Polyarteritis nodosa.
– Churg-Strauss syndrome.
– Relapsing polychondritis.
– Benign lymphocytic angitis.
– Nasopharyngeal lymphomas with pulmonary Hodgkin’s disease.
Allergic fungal sinusitis is a saprophytic fungal growth involving one or more paranasal sinuses. Allergic fungal sinusitis appears to be more prevalent in the temperate regions and around areas of high relative humidity with equal preponderance in both sexes.6 It can manifest itself either with signs and symptoms of nasal obstruction and allergic rhinitis or purulent rhinorrhoea, headache, and epistaxis. Orbital aspergillosis occurs as an extension of allergic fungal sinusitis into the adjacent spaces, which may have a dramatic clinical presentation such as diplopia or visual loss due to compression of the ophthalmic nerve. The gross facial disfigurement and orbital abnormalities consisting of proptosis and telecanthus are more often seen in children than in adults. The diagnosis of allergic fungal sinusitis is principally based on pathological findings in the specimens obtained from the paranasal sinuses. These findings are similar to those seen in the mucoid impaction of ABPA.6
Concomitant occurrence of ABPA and allergic fungal sinusitis is well recognised with substantial data available on the same.7 It has been postulated that the hallmark of this saprophytic colonisation is an identical pathophysiology occurring in ABPA, allergic fungal sinusitis, and orbital aspergillosis. There are several case reports of coexisting ABPA with allergic fungal sinusitis7 and allergic fungal sinusitis with orbital aspergillosis.6 The presence of ABPA and allergic fungal sinusitis in the same patient is often overlooked when either is being treated by two different specialties.
The management of allergic fungal sinusitis with orbital involvement warrants an immediate surgical removal of the fungal growth including resection of any recurring disease. Medical management includes the use of corticosteroids as in ABPA, while topical corticosteroids may be effective in controlling local inflammation. It is also recommended that systemic antifungals be used to prevent progression to invasive forms of fungal sinusitis. Intravenous amphotericin-B has been found to be more beneficial than the less toxic agents of the azoles group in view of the poor in vitro activity of these agents.6
In conclusion, patients with ABPA should be evaluated for the presence of coexistent allergic fungal sinusitis and orbital aspergillosis to avoid further complications.
Allergic fungal sinusitis with orbital aspergillosis in a patient with allergic bronchopulmonary aspergillosis.
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