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Introduction: Allergic bronchopulmonary mycoses are complex pulmonary disorders caused by immune reactions mounted against fungi, most often Aspergillus fumigatus (Allergic Bronchopulmonary Aspergillosis), which colonize the airways of patients with chronic lung disease, most commonly asthma, cystic fibrosis (CF), bronchiectasis and chronic obstructive pulmonary disease [1eur journal]. Although rare, other aspergillus spp.., such as A.flavus, A.niger, A.oryzae can also cause ABPA [2]. ABPA affects approximately 2% of patients with asthma. CASE: ABPA HAM AND ASPERGILLUS NIGER. A 32-year-old female presented with generalized weakness and fatigue for 6 months and dry cough for 3 months. She had multiple episodes of cough and shortness of breath in past 1 year, diagnosed as asthma and treated with short course inhaled corticosteroids and oral antibiotics.
CASE 1:
ABPA HAM AND ASPERGILLUS NIGER
A 32-year-old female presented with generalized weakness and fatigue for 6 months and dry cough for 3 months. She had multiple episodes of cough and shortness of breath in past 1 year, diagnosed as asthma and treated with short course inhaled corticosteroids and oral antibiotics.
The patient vitals were BP: 110/70 mmHg, PR: 95 /minute, SpO2: 97% at RA, RR: 24/min. On respiratory system examination, patient had bilateral expiratory polyphonic rhonchi, crepitations in right axillary and mammary areas.
HRCT chest showed consolidation and sub segmental atelectasis with bronchiectasis in the medial and lateral segments of right middle lobe (Figure 1) with hyper dense mucus plugging having an attenuation of 89HU (which is higher than the T4 para-spinal muscle of 70HU) (Figure 2).
Serology for aspergillus hypersensitivity was performed which revealed an elevated total IgE of 6853 IU/ml, elevated blood eosinophils (600cells/ dl), elevated aspergillus specific IgE 7.07kUA/L and an elevated aspergillus specific IgG 144mgA/L.
Flexible bronchoscopy was performed, broncho alveolar lavage (BAL) was collected from medial segment of right middle lobe and thick brownish mucus plug was aspirated. BAL cytology showed neutrophilic predominance (60%). Culture and sensitivity for pyogenic organisms was negative. Fungal culture showed growth of branching filamentous fungus. Lactophenol cotton blue (LPCB) mount showed large globose dark brown to black conidial heads with branched septate hyphae suggestive of A.niger complex (Figure 3a,3b). However here was no growth of A.fumigates.
Based on ISHAM diagnostic criteria, a diagnosis of Allergic broncho pulmonary Aspergillus fumigatus and Aspergillus niger was made.
Patient was started on oral prednisolone (0.5mg/kg/day and tapered over 3 months) and inhaled corticosteroids.
After 2 months of therapy serum total IgE showed over 25% decline (1843 IU/ml ,63% decline) and HRCT chest revealed the underlying middle lobe bronchiectasis after the HAM resolved with treatment (Figure 2).
DISCUSSION:
ABPA - HAM is a radiological phenotype of allergic bronchopulmonary aspergillosis. The presence of HAM on CT chest is 37% sensitive and 100% specific for ABPA [7]. Causative fungi for allergic bronchopulmonary mycosis (ABPM) are multifold. Aspergillus species, especially A.fumigatus , is most commonly isolated fungus. Although rare, A.niger is also implicated in causing ABPA in a few case reports. In the present case A.fumigatus serology was positive and fungal culture yielded A.niger, indicating a possibility of both the species being causative agents for ABPA. A possibility of cross reactivity between antigens of A.fumigatus and A.niger could not be excluded. In such a case, A.niger would be solely responsible for ABPA making it a rare clinical finding.
Image 1- Axial section of CT Chest showing high attenuated mucus (HAM – HU Mean: 106.340) in right middle lobe.
Image 2 – Axial sections of CT Chest after one month of treatment shows right middle lobe bronchiectasis.
Image 3a – Fungal stain with Lactophenol cotton blue (LPCB) mount showing black colonies of Aspergillus niger. A) Low magnification B) High magnification.
Image 3b – Fungal stain with Lactophenol cotton blue (LPCB) mount showing black colonies of Aspergillus niger. A) Low magnification B) High magnification.
CASE 2:
ABPA MUCOUS PLUG CAUSING MIDDLE LOBE COLLAPSE AND RESPIRATORY FAILURE WITH CO-EXISTANT PULMONARY TUBERCULOSIS
A 60-yr female, known asthmatic-GINA step II presented with history of low-grade fever with evening rise of temperature for 20 days, loss of appetite and weight (3 kgs in the past one month), cough with minimal sputum for 20 days. On examination patient had SpO2: 92% on room air, PR: 96/min, BP: 126/76mmHg, RR: 20/min. Chest X-ray showed a homogenous opacity obscuring the right heart border. HRCT chest (Figure 4) showed collapse consolidation of right middle lobe (with HU of 106). Flexible bronchoscopy was performed which revealed total occlusion of right middle lobe bronchus with a mucous plug (Figure 5a, 5b). The mucous plug is removed with the help of Fogarty’s balloon catheter and BAL was acquired. Figure 6 shows patent right middle lobe bronchus after removal of the mucous plug. BAL cytology showed 80% neutrophils, negative for malignant cells. BAL for GeneXpert MTB detected mycobacterium tuberculosis, rifampicin resistance not detected. Serology for ABPA revealed blood eosinophils =700/dl, aspergillus specific IgE 5.27 Kua/L (Normal is < 0.35 kUA/L), Aspergillus specific IgG 384 mgA/L (normal <27mgA/L). A diagnosis of ABPA- MP with pulmonary tuberculosis was made. Patient is started on ATT and oral corticosteroids starting at 0.5mg/kg tapered over 3 months. After 1 month Chest X-ray showed total resolution of right middle lobe collapse (Figure 7).
DISCUSSION:
Mucous plugs within the airways are seen commonly in ABPA, asthma and plastic bronchitis and less commonly in Aspergillus tracheobronchitis, Hyper-IgE syndrome, exogenous lipoid pneumonia, pulmonary alveolar proteinosis and chronic eosinophilic pneumonia [8].
Mucus plugs can partially or completely obstruct one or more airways and cause grave consequences, including atelectasis and recurrent infection. While reviewing 1340 chest radiographs in 113 patients with ABPA, lobar/segmental collapse was observed in 17% patients [9]. Mucus plugs in APBA leading to middle lobe collapse is uncommon. Chronic or recurrent collapse of middle lobe due to obstruction of middle lobe bronchus is called Middle Lobe Syndrome (MLS). It has now been recognized that several clinical entities can present as MLS, a few of them being described as case reports [10]. One among these uncommon presentations is the occurrence of an MLS in patients with ABPA. The non-obstructive benign form is typically caused by inflammation, accounting for around 62% of cases and has been identified as the most common cause of MLS. [11]
Presenting usually as chronic respiratory illness, only rarely does it manifest as acute respiratory failure caused by unilateral lung collapse.
Coexistence of ABPA and tuberculosis has been reported earlier in a few case reports. In a report by Kim et al, diagnosis of tuberculosis was achieved after lobectomy in a patient recently diagnosed as ABPA. Saurabh Mittal et. al. also reported, coexistent ABPA and tuberculosis presenting as lung collapse with respiratory failure [12].
The probable hypothesis for this is imbalance between Th1 and Th2 lymphocytes driven inflammation. This swaying of immune system towards Th2 inflammation may probably increase the susceptibility towards mycobacterial infection.
APBA presents with the constitutional symptoms like weight loss, fatigue, and mass-like infiltrates on chest imaging. This presentation can easily be confused for malignancy or pulmonary tuberculosis. Mucus plugs usually involve upper lobes in ABPA, lower lobes in plastic bronchitis [8].
This is one of the very few cases in the literature describing coexistent ABPA with tuberculosis presenting as respiratory failure.
Image 4 – Axial section of CT Chest showing right middle lobe consolidation with collapse
Image 5a – Bronchoscopic image of right middle lobe with mucus plug within.
Image 5b – Bronchoscopic image of right middle lobe with mucus plug within.
Image 6 – Bronchoscopic image of right middle lobe completely patent after removal of the mucous plug.
Image 7 - Chest X rays showing complete resolution of opacity after 1 month of therapy with oral corticosteroids
CASE 3:
ABPA CENTRAL AND PERIPHERAL BRONCHIECTASIS
A 41-year-old female presented with complaints of progressive exertional breathlessness, cough with mucoid sputum and wheezing for the past three months. Patient had smear positive pulmonary tuberculosis treated adequately 5years ago, using inhaled medications intermittently for symptom relief since then.
Patient vitals were as follows - PR: 86 bpm, SpO2: 98% at RA, BP: 110/80 mmHg, RR: 16/min. On examination patient had bilateral expiratory polyphonic rhonchi, coarse crepitations in bilateral infra-scapular, inter-scapular, mammary and axillary areas.
Blood investigations revealed – Hb: 11.5gm%, WBC count: 10,800cells/dl
HRCT chest showed dilated air spaces with thickened wall filled with mucus and extensive cystic bronchiectasis in both lungs (Figure 8a, 8b).
Serology for aspergillosis hypersensitivity revealed an elevated total serum IgE of 1607KIU/L, eosinophilia count of 648cells/cumm, Aspergillus specific IgE of 26.50KUA/L, Aspergillus specific IgG of 2.33 mgA/L. These findings are consistent with ISHAM criteria for ABPA.
She was started on oral prednisolone 0.5mg/kg/day and tapered over 3months. Patient showed significant improvement in symptoms after 2months. Repeat serum IgE showed over 25% decline (915.4 KIU/L,43% decline).
DISCUSSION:
Central bronchiectasis is the hallmark of ABPA. bronchiectasis in ABPA can extend to the periphery in up to 40% of the lobes [1]. ABPA may complicate asthma, thus leading to bronchiectasis [13]. Aspergillus can complicate existing bronchiectasis with post infective bronchiectasis the being most common.
The retention of Aspergillus allergens in already established bronchiectatic airways may help drive ABPA. However, it is also possible that Aspergillus proteases in normal (or asthmatic/COPD) air ways in susceptible individuals drive airway inflammation toward frank bronchiectasis through up regulating of mucus production and/or driving a Th2 phenotype [14][15]. A.fumigatus can lead to invasive aspergillosis, defined as histologic evidence of tissue invasion by branched septate hyphae.
While invasive Aspergillus infections are well-recognized complications in those with immunosuppression, this serious and life-threatening infection is also well described in those with underlying chronic lung diseases such as asthma, emphysema, and bronchiectasis, particularly in the setting of oral corticosteroid use [13].
A.niger is less rapidly growing than A.fumigatus. Unlike A.niger, Proteases released by A.fumigatus are very potent, even with few organisms' present explaining why A.fumigatus causes ciliary dyskinesia incredibly early [13].
Image 8a: axial sections of CT chest showing multiple cystic air spaces in both central and peripheral areas of lung.
Image 8b: axial sections of CT chest showing multiple cystic air spaces in both central and peripheral areas of lung.
CASE 4:
POST TUBERCULAR FIBRO-CAVITORY DISEASE WITH ABPA-CPF (ABPA-Chronic pleuropulmonary fibrosis)
A 69-year-old male, current smoker (more than 30 pack years) presented to Emergency room with shortness of breath. He was tachypnoeic at rest with BP of 110/70mmHg, pulse rate of 105/min, saturation of 88% at RA and RR of 24/ min. On respiratory system examination, patient had bilateral expiratory wheeze, crepitations in right infra scapular and infra-axillary area and tubular bronchial breathing in left infra scapular area. ABG showed type 2 respiratory failure. Patient is diagnosed as COPD with acute exacerbation.
Patient had history of pulmonary tuberculosis 4years back. He had multiple outpatient visits and four admissions with COPD exacerbations.
HRCT chest showed bilateral upper lobe cavities with multiple cystic airspaces,areas of paraseptal emphysema diffusely distributed in both the lungs along with pleural thickening with fibrotic bands in the right lung (Figure 9a, 9b, 10a, 10b).
Owing to frequent COPD exacerbations, patient is evaluated for ABPA.
Blood investigations revealed a WBC count of 8,600 cells/cumm, absolute eosinophilic count of 648cells/cumm, serum IgE of 2435IU, Aspergillus specific IgE of 0.49kUA/L and Aspergillus specific IgG of 34.5mgA/L. Sputum smear for AFB and Genexpert was negative. A diagnosis of ABPA-CPF has been made and started on oral prednisolone 0.5mg/kg/day to be tapered over 3 months.
DISCUSSION:
Patients with history of pulmonary tuberculosis are known to have a large number of complications including COPD, bronchiectasis, persistent lung cavities and chronic pulmonary aspergillosis. However other than a few sporadic cases, the occurrence of ABPA has rarely been reported in these patients.
In a study of 1012 patients with post tubercular sequelae by Animesh Ray, 24 (2.37%) patients had ABPA [16].
In a cross-sectional study by Gurav P et al., 165 asthma patients were screened for ABPA, 42 of them had ABPA, and 10(23.8%) had ABPA-CPF [17].
Revised ISHAM- AWG recommendations for APBA-CPF include two or more of the following - pulmonary fibrosis, fibro-cavitary lesions, fungal ball, and pleural thickening [18]. Other radiological findings include calcific mediastinal lymph nodes and cicatricial emphysema.
At this stage, distinguishing ABPA from old pulmonary tuberculosis is difficult.
Image 9a: Axial sections of CT chest showing multiple areas of emphysema bilaterally.
Image 9b
Image 10a: Axial sections of CT Chest showing areas of fibrosis and pleural thickening in bilateral lungs.
Image 10b: Axial sections of CT chest showing areas of pleural thickening and cavities in bilateral lungs.
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