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CASE REPORT
tion tests demonstrated a mildly ele- Bronchiolitis oblit- vated alanine transaminase (66 IU/l), aspartate transaminase (79 IU/l) and lactate dehydrogenase (284 IU/l). erans organising The rheumatoid factor and anti- nuclear factor were negative. pneumonia (BOOP) Serological tests for Legionnaire’s dis- ease, mycoplasma and chlamydia were or cryptogenic negative. Sputum examination revealed no organisms on gram-stain organising pneumo- and culture. Thoracentesis demon- strated a transudate. nia (COP) The lung function test showed a moderate restrictive pattern. The chest X-ray demonstrated 1-3 VKBhagwandas is found. We report on a patient bilateral basal infiltrates with blunting recently seen with this condition. MB BCh, FCRad (D) (SA) of both costophrenic angles suggest-
X-ray Department ing pleural effusions (Fig. 1). Lenmed Clinic Case report Lenasia Johannesburg A 36-year-old woman presented with a history of persistent non-pro- R Morar ductive cough for 4 weeks. She com- MB ChB, MMed, FCP (SA), FCCP plained of pleuritic chest pain, night Department of Pulmonology sweats and progressive shortness of University of the Witwatersrand Johannesburg Hospital breath with decreased effort tolerance, and she remained symptomatic HDBhula despite numerous antibiotics. There BSc (Micro), MB BS (Bombay), FCP (SA) were no contributing factors in the Fig. 1. Bibasal infiltrate with blunting of the Lenmed Clinic history to elucidate the aetiology of costophrenic angles suggesting effusions. Lenasia her symptoms. There was no history Johannesburg of previous allergies, drug use, recent upper respiratory tract infection, sig- Despite intravenous antibiotics Introduction nificant dust exposure, radiation ther- there was no clinical response. High- resolution computerised tomography Bronchiolitis obliterans organising apy, transplantation or autoimmune (HRCT) scan of the chest was then pneumonia (BOOP) is a distinct enti- disease. performed which demonstrated ty with various clinical, radiographic Systematic examination revealed a patchy bilateral air space consolida- and prognostic features. Patholo- young female in respiratory distress, tion involving predominately the gically it is characterised by the pres- with decreased breath sounds and fine lower lobes. This consolidation ence of granulation tissue polyps inspiratory crackles at both bases. The appeared to be subpleural and peri- within the lumina of bronchioles and cardiovascular system and the rest of bronchovascular in distribution. alveolar ducts with patchy areas of the examination were normal. Small bilateral pleural effusions were organising pneumonia. Chronic The blood results revealed mild also confirmed (Fig. 2). There was evi- inflammation is noted in the walls of microcytic hypochromic anaemia. dence of patchy ground-glass opacifi- the surrounding alveoli with pre- The C-reactive protein and erythro- cation in the lower lobes. The diagno- served lung architecture. The pre- cyte sedimentation rate (ESR) were sis of atypical pneumonia and BOOP ferred term is cryptogenic organising within normal limits. The urea and was considered. pneumonia (COP) when no aetiology electrolytes were normal. Liver func-
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CASE REPORT
Idiopathic BOOP The clinical presentation of idio- pathic BOOP,which is the most com- mon type encountered, and the type present in our patient, is one of flu- like illness with fever, mild cough and dyspnoea. Haemoptysis is uncom- mon. Bilateral basal crackles are Fig. 2. Patchy bibasal consolidation with subpleural and peribronchovascular distribution. noted. Pulmonary function tests demonstrate a restrictive pattern and abnormal diffusing capacity. The ESR is usually raised.1-3 Chest X-ray findings may include unilateral or bilateral patchy alveolar airspace consolidation which is sub- pleural and peribronchial in location, usually in the lower regions of each lung. The consolidation may be mul- tifocal and non-segmental or unilater- Fig. 3. Interstitial thickening with lymphocytes and plasma cells with granulation tissue type exudates and fibroblast proliferation filling the alveolar spaces. al and focal. Nodules 3 - 5 mm in size and other linear opacities may also be An open lung biopsy of the lingu- seen. Pleural thickening and/or effu- la and left lower lobe segments sions may also be noted.1, 3 revealed interstitial thickening with HRCT scans may show bilateral lymphocytes and plasma cells, with areas of consolidation and ground- granulation tissue-type exudates and glass opacities are seen mainly in a fibroblast proliferation filling the alve- peripheral location.4 Two types of olar spaces (Fig. 3). There was no evi- linear opacities may be noted. The dence of tuberculosis, sarcoidosis or first extends in a radial manner along malignancy. Multiple sections exam- the line of the bronchi toward the ined revealed features in keeping with pleura, and the second occurs in the subpleural location with no relation BOOP. Fig. 4. Significant resolution of bibasal infil- Following confirmation of the trates post treatment. to bronchi. Both types usually occur diagnosis of idiopathic BOOP, treat- in the lower lobes, frequently associ- ment with corticosteroids was com- with usual interstitial pneumonitis / ated with multifocal areas of consoli- menced at a dose of 0.5 mg /kg. A fol- idiopathic pulmonary fibrosis (UIP/ dation.5 Centrilobular nodules mea- low-up chest X-ray obtained 3 weeks IPF).2 BOOP may be classified as fol- suring 3 - 5 mm in diameter may be post treatment (Fig. 4) showed signif- lows1-3:(i) idiopathic; (ii) rapidly pro- observed. Bronchial wall thickening icant resolution of the bilateral basal gressive; (iii) focal nodular; (iv) multi- and cylindrical bronchial dilation may infiltrates. ple nodular; (v) post infection; (vi) also be noted. Small ill-defined nodu- drug related; (vii) autoimmune dis- lar opacities of 1 - 10 mm in diameter Discussion ease; (viii) bone marrow transplanta- may also be seen. Pleural effusions tion; (ix) lung transplantation; (x) may be present.4,5 Cavitating lung BOOP or COP is an inflammato- renal transplantation (xi) radiothera- masses are rare.6 The differential diag- ry lung disease and thus is related to py; (xii) environment-related; (xiii) nosis includes eosinophilic pneumo- the inflammatory pathway rather miscellaneous. nia, pulmonary haemorrhage, multi- than the fibrosing pathway that occurs
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CASE REPORT
focal infection and diffuse alveolar spaced rounded balls of myxomatous The prognosis is usually good, except damage.4-8 connective tissue present.1-3 in a rapidly progressive form of this Magnetic resonance imaging The diagnosis of BOOP/COP is condition.1, 3 (MRI) currently has no significant definite in a patient with a typical References diagnostic role in BOOP, but may pathologic presentation on a lung 1. Cordier JF. Cryptogenic organizing pneumo- have a role to play in the follow-up of biopsy of sufficient size. However, nia. Clin Chest Med 2004; 25: 727-738, vi-vii. patients with BOOP.It may be used to transbronchial lung biopsy (smaller 2. American Thoracic Society; European Respiratory Society. American Thoracic assess the response to treatment and samples) in a patient with typical clin- Society/European Respiratory Society International Multidisciplinary Consensus disease activity. Broncho-alveolar car- ico-radiological presentation may Classification of the Idiopathic Interstitial cinoma may mimic BOOP.The white suggest the diagnosis of BOOP. Pneumonias. This joint statement of the American Thoracic Society (ATS), and the lung sign is an uncommon finding in Conditions that mimic BOOP European Respiratory Society (ERS) was adopt- pulmonary consolidation evaluated include pulmonary lymphoma, ed by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. with heavily T2-weighted sequences. chronic eosinophilic pneumonia, Am J Respir Crit Care Med 2002; 165: 277-304. This sign is usually negative in broncho-alveolar carcinoma and pul- 3. Epler GR. Bronchiolitis obliterans organising pneumonia. Arch Intern Med 2001; 161: 158- patients with BOOP but positive in monary embolism. 164. patients with broncho-alveolar carci- It is important that the classified 4. Webb WR, Muller NL, Naidich DP. High- Resolution CT of the Lung. 3rd ed. Philadelphia: noma.9 Therefore MRI has a potential aetiological factors related to BOOP Lippincott-Raven, 2001: 373-378, 529-537. role to play in the exclusion of other be excluded from the history, exami- 5. Murphy JM, Schnyder P, Verschakelen J, Leuenberger P, Flower CD. Linear opacities on conditions. nation and investigations, especially HRCT in bronchiolitis obliterans organising Ultrasound may assist in the char- infection, drug or radiation-induced pneumonia. Eur Radiol 1999; 9: 1813-1817. 6. Akira M, Yamamoto S, Sakatari M. acterisation of pleural effusions and pulmonary reaction, autoimmune Bronchiolitis obliterans organising pneumonia manifesting as multiple large nodules or mass- for the guidance of pleurocentesis. diseases and systemic inflammatory es. Am J Roentgenol 1998; 170: 291-295. The gold standard for confirming disease. When no aetiology is found, 7. Muller N, Staples C, Miller R. Bronchiolitis obliterans organizing pneumonia: CT features the diagnosis is a lung biopsy either by as in our patient, the organising pneu- in 14 patients. Am J Roentgenol 1990; 154: 983- video-assisted thoracoscopic surgery monia may be called cryptogenic. 987. 8. Nishimura K, Itoh H: High-resolution comput- (VATS) or open lung biopsy. This condition is a form of idiopathic ed tomographic features of bronchiolitis oblit- Histologically there is evidence of interstitial pneumonitis. erans organizing pneumonia. Chest 1992; 102: 26S-31S. intraluminal organising and polypoid Finally, treatment for this condi- 9. Murphy J, Schnyder P, Herold C, Flower C. granulation tissue within small bron- tion is usually prolonged corticos- Bronchiolitis obliterans organising pneumonia simulating bronchial carcinoma. Eur Radiol chioles, alveolar ducts and alveoli. teroid administration for approxi- 1998; 8: 1165-1169. There are also foamy macrophages, mately 6 - 12 months in tapering inflamed alveolar walls and evenly doses depending on clinical response.
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