Rapidly progressive Bronchiolitis Obliterans Organising Pneumonia presenting with pneumothorax, persistent air leak, acute respiratory distress syndrome and multi-organ dysfunction: a case report
Rapidly progressive Bronchiolitis Obliterans Organising Pneumonia presenting with pneumothorax, persistent air leak, acute respiratory distress syndrome and multi-organ dysfunction: a case report
Journal of Medical Case Reports 2008, 2:145
Published: December 2009
Published: December 2009
Abstract
Introduction
Bronchiolitis Obliterans Organising Pneumonia (BOOP) may often present initially as a recurrent spontaneous pneumothorax and then develop multi-system complications.
Case presentation
A 17-year-old boy presented with a pneumothorax, which developed into rapidly progressive Bronchiolitis Obliterans Organising Pneumonia (BOOP). He developed multi-organ dysfunction (including adult respiratory distress syndrome, oliguric renal failure, acute coronary syndrome, cardiac failure and a right atrial thrombus) which necessitated prolonged intensive care. Diagnosis was confirmed on open lung biopsy and he responded well to treatment with corticosteroids.
Conclusion
BOOP is exquisitely sensitive to oral corticosteroids but if the diagnosis is not considered in such patients and appropriate treatment instituted early, BOOP may often lead to prolonged hospital admission with considerable morbidity.
Introduction
Bronchiolitis Obliterans Organising Pneumonia (BOOP) is a clinico pathological entity [1] characterised histologically by polypoid masses of granulation tissue in the lumen of small airways, alveolar ducts and alveoli co existing with fibrosis [2]. Its distribution is patchy, with preservation of background architecture [1]. Lung function tests demonstrate impaired diffusion capacity with a restrictive picture [3] while the radiological appearance is of patchy consolidation and alveolar infiltrates in a peri-bronchiolar or pleural distribution [4]. We report on what we believe to be the first case of BOOP associated with persistent air leak, multiple organ failure and acute coronary syndrome.
Keywords:
Bronchiolitis Obliterans syndrome, Bronchiolitis Obliterans, diffuse panbronchiolitis,bronchiolitis oblitereans, pneumothorax, pneumothorax chest pain, collapsed lung pneumothorax, cystic fibrosis pneumothorax, Pneumonia treatment, Pneumonia, aspiration Pneumonia, Pneumonia symptoms, Pneumonia contagious, types of Pneumonia, hypothermia,
Bronchiolitis Obliterans syndrome, Bronchiolitis Obliterans, diffuse panbronchiolitis,bronchiolitis oblitereans, pneumothorax, pneumothorax chest pain, collapsed lung pneumothorax, cystic fibrosis pneumothorax, Pneumonia treatment, Pneumonia, aspiration Pneumonia, Pneumonia symptoms, Pneumonia contagious, types of Pneumonia, hypothermia,
References:
- Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA: Bronchiolitis obliterans organizing pneumonia. N Engl J Med 312(3):152-8. 1985 Jan 17
- Myers JL, Katzenstein AL: Ultrastructural evidence of alveolar epithelial injury in idiopathic bronchiolitis obliterans-organizing pneumonia. Am J Pathol 1988, 132(1):102-9.
- Muller NL, Guerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC: Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology 1987, 162(1 Pt 1):151-6.
- Muller NL, Staples CA, Miller RR: Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR Am J Roentgenol 1990, 154(5):983-7.
- Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD: Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 157(12):1323-9. 1997 Jun 23
- Epler GR: Drug-induced bronchiolitis obliterans organizing pneumonia. Clin Chest Med 2004, 25(1):89-94.
- Mokhtari M, Bach PB, Tietjen PA, Stover DE: Bronchiolitis obliterans organizing pneumonia in cancer: a case series. Respir Med 2002, 96(4):280-6.
- Poletti V, Cazzato S, Minicuci N, Zompatori M, Burzi M, Schiattone ML: The diagnostic value of bronchoalveolar lavage and transbronchial lung biopsy in cryptogenic organizing pneumonia. Eur Respir J 1996, 9(12):2513-6.
- Perez de Llano LA, Soilan JL, Garcia Pais MJ, Mata I, Moreda M, Laserna B: Idiopathic bronchiolitis obliterans with organizing pneumonia presenting with adult respiratory distress syndrome. Respir Med 1998, 92(6):884-6.
- Patel SR, Karmpaliotis D, Ayas NT, Mark EJ, Wain J, Thompson BT, Malhotra A: The role of open-lung biopsy in ARDS. Chest 2004, 125(1):197-202.
- Kramer MR, Berkman N, Mintz B, Godfrey S, Saute M, Amir G: The role of open lung biopsy in the management and outcome of patients with diffuse lung disease. Ann Thorac Surg 1998, 65(1):198-202.
- Kofteridis DP, Bouros DE, Vamvakas LN, Stefanaki KS, Voludaki AE, Barbounakis EM, Emmanouel DS: Pneumothorax complicating fatal bronchiolitis obliterans organizing pneumonia. Respiration 1999, 66(3):266-8.
- Iwanaga T, Hirota T, Ikeda T: Air leak syndrome as one of the manifestations of bronchiolitis obliterans organizing pneumonia. Intern Med 2000, 39(2):163-5.
- Yang DG, Kim KD, Shin DH, Choe KO, Kim SK, Lee WY: Idiopathic bronchiolitis obliterans with organizing pneumonia presenting with spontaneous hydropneumothorax and solitary pulmonary nodule. Respirology 1999, 4(3):267-70.
- Nizami IY, Kissner DG, Visscher DW, Dubaybo BA: Idiopathic bronchiolitis obliterans with organizing pneumonia. An acute and life-threatening syndrome. Chest 1995, 108(1):271-7.
add new comment Comments