å¡ CASE REPORT å¡

Air Leak Syndrome as One of the Manifestations of Obliterans Organizing Tomoaki Iwanaga, Takako Hirota and Togo Ikeda

Abstract prior to hospital admission when he developed a persistent cough. He was treated with antitussives and bronchodilators A 46-year-old man developed respiratory distress with without complete resolution of his symptoms. Four days prior air leak syndrome (ALS), including , pneu- to admission, he developed moderate respiratory distress asso- momediastinum,and .Openlung ciated with a severe dry cough and low-grade fever. He had no biopsy was performed and revealed the histopathologic known allergies and no history of smoking. evidence of bronchiolitis obliterans organizing pneumonia Physical examination revealed a temperature of 37.7°C (BOOP), which responded well to steroid treatment. As far orally, a heart rate of 126 beats/min, a respiration rate of 24/ as we know, this appears to be the first case ofBOOPpre- min, and massive subcutaneous emphysemaextending over senting with ALSas one of its major complications. the chest and upper abdomen. Initial laboratory studies showed (Internal Medicine 39: 163-165, 2000) a WBCcount of 1 3,490/jil with 10% eosinophils. Arterial blood gas analysis while breathing 5 //min of oxygen showed a pH of Key words: pneumothorax, subcutaneous emphysema, pneu- 7.42, a PCO2of42, and a PO2 of65. Blood and sputum cul- momediastinum, open biopsy tures, and Gramstaining and acid-fast preparations of the spu- tum were negative. The (Fig. 1 , upper) showed bibasilar infil- trates and prominent subcutaneous emphysema.Chest CTscan- Introduction ning (Fig. 1 , lower) demonstrated bilateral airspace consolida- tion, limited pneumothorax, pneumomediastinum, and subcu- Idiopathic bronchiolitis obliterans with organizing pneumo- taneous emphysema.Bronchoscopy was performed shortly after nia (BOOP)is a clinicopathologic syndrome first proposed by admission. Histologic examination of the transbronchial biopsy Epler et al (1) which is characterized by an indolent clinical specimens revealed inflammatory infiltrates with focal fibro- course and a favorable prognosis, although a fulminating vari- sis. Bronchoalveolar lavage fluid from the upper lobe of the ant has been documented (2). BOOPfeatures the ingrowth of right lung showed an increase in lymphocytes (54%) with a polypoid fibroinflammatory granulation tissue from the bron- decrease in the CD4/CD8 ratio (0.29). chioles into the adjacent alveoli where organizing pneumonia Open lung biopsy was performed on the left upper and lower forms. The typical patient presents with dyspnea, cough, fe- lobes revealing multiple foci of myxomatousfibrous tissue ver, weight loss and single or multiple alveolar opacities on obliterating the distal air spaces and associated with an inter- the chest radiograph. Air leak syndrome (ALS), comprising stitial inflammatory infiltrate (Fig. 2). These findings and the pneumothorax, pneumomediastinum,and subcutaneous em- bronchoalveolar lavage (BAL) data were indicative of BOOP. physema, has never previously been reported as part of the Noneof the serologic parameters or tissue cultures for evalua- presentation of BOOP.Here we report a case of BOOPin which tion of infectious causes werepositive. Therapywasstarted ALSwas the major presenting symptom. with 40 mgper day of prednisolone and there was gradual im- provement of his symptoms. His ALSdisappeared without any Case Report specific interventions including chest tube insertion. The pred- nisolone dose was progressively tapered and he showeda sat- A 46-year-old manwas hospitalized after presenting with isfactory course. dry cough, respiratory distress, and prominent subcutaneous emphysema. He was in his usual state of health until 4 months

From the Department of Pulmonary Medicine, National Minami-Fukuoka Chest Hospital, Fukuoka Received for publication August 12, 1999; Accepted for publication October 23, 1999 Reprint requests should be addressed to Dr. Tomoaki Iwanaga, the Department of Pulmonary Medicine, National Minami-Fukuoka Chest Hospital, 4-39-1, Yakatabaru, Minami-ku, Fukuoka 8 1 1 - 1 394

Internal Medicine Vol. 39, No. 2 (February 2000) 163 Iwanaga et al

Figure 2. Histologic specimen from the left lung revealing multiple foci of myxomatous fibrous tissue obliterating the dis- tal air spaces, accompanied by a chronic interstitial inflamma- tory infiltrate and slight fibrosis in the alveolar septa (HE stain, x200).

adjacent tissues, will favor alveolar rupture (4). In parenchy- mal lung diseases, overexpansion of the distal air spaces be- yond sites of small leads to alveolar rup- ture triggered by coughing or straining (5). Once it reaches the , air tends to spread throughout the into the subcutaneoustissues. The hallmark pathologic change of BOOPis the presence of granulation tissue plugs within the lumens of the distal bron- chioles extending into the alveolar ducts and alveoli (1). Al- thoughlung function studies showno airflow obstruction ex- cept in smokers (6), localized or regional peripheral obstruc- tion can result in a ball-valve effect and distal overdistension leading to burst alveoli and entry of air into the bronchovascular sheath followed by manifestation of any form of aberrant air trapping, orALS. The present patient had a severe cough which might have caused overpresssurization of the alveoli. Though Figure 1. A chest radiograph (upper) and a CTscan of chest cough and dyspnea are commonclinical features of BOOP(7), (lower) showing bilateral pulmonary infiltrates as well as mas- there has been no mention of severe cough causing ALS.To sive subcutaneous emphysema, limited pneumothorax, and pneu- momediastinum. our knowledge, ALShas not previously been described as the cardinal presenting manifestation of this disease and this ap- pears to be the first case of a BOOPpatient with prominent ALS. Discussion Since ALS, pulmonary infiltrates, and eosinophilia of the peripheral blood and sputum were the predominant presenting ALS, comprising pneumothorax, pneumomediastinum, and features in the present patient, was a subcutaneous emphysema,has been described as a complica- possible alternate diagnosis. However, lymphocytosis with a tion of manylung diseases. The most commondisorders un- decreased CD4/CD8ratio in the BALfluid made this possibil- derlying secondary ALSare chronic obstructive pulmonary ity unlikely and compatible with BOOP(8), which was con- disease and , in which rupture of bullae occurs. For ex- firmed by open lung biopsy. The possibility of asthma was not ample, pneumomediastinumis a well- described complication likely because of lack of air flow obstruction. Currently, no of acute asthma, having been noted in 5.4% of 479 chest X-ray consensus exists regarding the optimumdose or duration of films of children admitted with asthma attacks (3). However, corticosteroid therapy for BOOP.Our patient responded gradu- anything that produces alveolar overdistension, or a momen- ally to intravenous prednisolone with an initial dose of 40 mg tary discrepancy between the alveolar pressure and that in the a day and slow tapering over several months. Since his pneu- 164 Internal Medicine Vol. 39, No. 2 (February 2000) Air Leak Syndrome Associated with BOOP mothorax was limited, ALSdisappeared without any specific 2) Nizami IY, Kisser DG, Visscher DW, Dubaybo BA. Idiopathic bronchi- therapy. olitis obliterans with organizing pneumonia. An acute and life-threaten- ing syndrome. Chest 108: 271-277, 1995. Although the clinical and radiological features of BOOPare 3) Eggleston PA, Ward BH, Pierson WE, Bierman CW. Radiographic ab- often distinctive, it may be masked by an unusual presentation normalities in acute asthma in children. Pediatrics 54: 442-449, 1974. such as ALSin the present case. Weconclude that BOOPshould 4) Joannides M, Tsoulsos GD. The etiology of interstitial and mediastinal emphysema. Arch Surg 21: 333-339, 1930. be included in the differential diagnosis of patients presenting 5) Schaefer KE, McNulty WPJr, Carey C, et al. Mechanisms in develop- with ALS. Open lung biopsy for confirmation of the diagnosis mentof interstitial emphysemaand air embolismon decompression from is important, because BOOPusually shows a good response to depth. JAppl Physiol 13: 15-29, 1958. corticosteroids, as was noted in our patient. 6) Epler GR. Bronchiolitis obliterans organizing pneumonia: definition and clinical features. Chest 102 (Suppl): 2S-6S, 1992. Refe re nces 7) King TEJr, Mortenson RL. Cryptogenic organizing : the North American experience. Chest 102 (Suppl): 8S-13S, 1992. 1) Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA. Bron- 8) Yamamoto M, Ina Y, Kitaichi M, Harasawa M, Tamura M. Bronchiolitis chiolitis obliterans-organizing pneumonia. N Engl J Med 312: 152-158, obliterans organizing pneumonia (BOOP)in Japan. Jpn J Chest Dis 28: 1985. 1164-1173, 1990.

165 Internal Medicine Vol. 39, No. 2 (February 2000)