REFRACTORY ASTHMA IN A 14 YEAR OLD ASIAN MALE

Abstract

Introduction Diffuse panbronchiolitis (DPB) is a rare inflammatory disease of the lung with poor prognosis if left untreated. It is a primarily Th1 driven process with involvement of CD8+ lymphocytes and neutrophils driving inflammation in the respiratory tract. Case Description A 14-year-old male of Asian descent with history of well-controlled asthma presented with worsening productive cough and persistent wheezing despite escalation of asthma therapy to high dose ICS/LABA (Advair 230/21 2 puffs BID). Spirometry showed persistent and worsening obstruction: FEV1/FVC = 70% with no improvement post bronchodilator administration. Chest CT showed diffuse ground glass centrilobular opacities along with multiple pulmonary nodules. BAL culture was positive for Haemophilus influenzae and Aspergillus terreus. Given the clinical course and CT findings, a diagnosis of diffuse panbronchiolitis was suspected and the patient was started on Azithromycin 250 mg daily. Repeat HRCT Chest 6 weeks later showed interval improvement in opacities seen previously. Macrolide therapy was continued for 9 months and repeat HRCT after completion of therapy showed resolution of previous pathologic lung findings. Clinically, the patient was reportedly asymptomatic and had normal lung function on testing. Conclusion Our patient's presentation and clinical course is unique in that after years of well-controlled asthma, he develops a rather acute onset of worsening pulmonary function, which prompted his workup and subsequent diagnosis with DPB. Our case suggests the importance of having DPB on the differential in severe asthmatics that are refractory to escalating treatment. Early diagnosis of DPB is essential for prompt Macrolide therapy. Diffuse panbronchiolitis (DPB) is a rare inflammatory disease of the lung with poor prognosis if left untreated. It is a primarily Th1 driven process with involvement of CD8+ lymphocytes and neutrophils driving inflammation in the respiratory tract. A 14-year-old male of Asian descent with history of well-controlled asthma presented with worsening productive cough and persistent wheezing despite escalation of asthma therapy to high dose ICS/LABA (Advair 230/21 2 puffs BID). Spirometry showed persistent and worsening obstruction: FEV1/FVC = 70% with no improvement post bronchodilator administration. Chest CT showed diffuse ground glass centrilobular opacities along with multiple pulmonary nodules. BAL culture was positive for Haemophilus influenzae and Aspergillus terreus. Given the clinical course and CT findings, a diagnosis of diffuse panbronchiolitis was suspected and the patient was started on Azithromycin 250 mg daily. Repeat HRCT Chest 6 weeks later showed interval improvement in opacities seen previously. Macrolide therapy was continued for 9 months and repeat HRCT after completion of therapy showed resolution of previous pathologic lung findings. Clinically, the patient was reportedly asymptomatic and had normal lung function on testing. Our patient's presentation and clinical course is unique in that after years of well-controlled asthma, he develops a rather acute onset of worsening pulmonary function, which prompted his workup and subsequent diagnosis with DPB. Our case suggests the importance of having DPB on the differential in severe asthmatics that are refractory to escalating treatment. Early diagnosis of DPB is essential for prompt Macrolide therapy.