Case Report Autopsy Findings of Fatal Cryptogenic Organizing Pneumonia

Home , Bronchopneumonia, Pleural effusion

Int J Clin Exp Pathol 2013;6(6):1128-1131 www.ijcep.com /ISSN:1936-2625/IJCEP1209031

Case Report Autopsy findings of fatal cryptogenic organizing pneumonia

Tadashi Terada

Department of Pathology, Shizuoka City Shimizu Hospital, Shimizu, Shizuoka, Japan Received September 29, 2012; Accepted March 25, 2013; Epub May 15, 2013; Published June 1, 2013

Abstract: Autopsy cases of cryptogenic organizing pneumonia (COP) have been rarely reported. A 73-year-old Japanese man consulted to a hospital because of flu-like sickness. He was diagnosed as pneumonia, and treated by antibiotics. He was referred to our hospital for further treatment. Chest X-P showed pneumonia involving the whole lungs. Blood laboratory test showed leukocytosis, increased CRP, and decreased PaO2. Despite of steroid therapy, he showed a downhill course and died one month after the first manifestation. The clinical diagnosis was acute pneumonia or ARDS. At autopsy, the both lungs were voluminous. The weight of lungs was 1050 g in the left lung and 1300 g in the right lung. The both lungs were entirely affected. The lungs were hard and little air was recognized. Microscopically, almost all alveolar spaces contained Masson’s bodies. Bronchiolitis obliterans was not recognized. The alveolar walls were not affected. The Masson’s bodies showed collagenization with lymphocytic infiltration. Hyalinization of Masson’s bodies with little inflammatory infiltration was frequently seen. Cartilagenous metaplasia and ossification of Masson’s bodies were seen in some places. The pulmonary arteries were affected by fibrosis, and occasionally showed thrombosis. The pathological diagnosis was COP. The heart weighted 500 g, and showed right ventricular hypertrophy (cor pulmonale). Other pathologic changes were pleural effusion (left, 800 ml: right, 1200 ml), acute liver congestion, prostatic hypertrophy, colon adenoma, and hypercellular bone marrow. The cause of death was respiratory failure due to COP and pleural effusion. In conclusion, the author reported an autopsy case of fatal COP.

Keywords: Lung, cryptogenic organizing pneumonia, histopathology

Introduction Case report

Focal organizing pneumonia is frequently seen A 73-year-old Japanese man consulted to a in patients with bronchopneumonia. However, hospital because of flu-like sickness. He was diffuse organizing pneumonia without features diagnosed as pneumonia, and treated by antibi- of acute bronchopneumonia was rare. In the otics. However, no improvement was obtained, past, such condition was called bronchiolitis and respiratory failure emerged. Therefore, he obliterans organizing pneumonia (BOOP) [1, 2]. was referred to our hospital for further treat- However, constrictive bronchiolitis obliterans ment. On admission, physical examination was similar to BOOP, and BOOP without bron- showed fever, cyanosis, and rale in both lungs. chiolitis obliterans was present. Therefore, dif- Chest X-P showed pneumonia involving the fuse idiopathic organizing pneumonia without whole lungs. Blood laboratory test showed leu- bronchiolitis obliterans was called cryptogenic kocytosis (28,500 /μl), increased CRP (29.7 organizing pneumonia (COP) [1, 2]. COP is very mg/dl), and decreased PaO2 (27.5 mmHg). No rare. Patients with COP show relatively well organisms were obtained from the sputum and prognosis [1-4]. Steroid is very effective in COP blood. No eosinophilia was noted. He showed [1-4]. Fatal COP is very rare, and there have no collagen diseases, and autoantibodies were been few reports of autopsy cases of fatal COP. all negative. No causes of the pneumonia were The author herein reports a case of fatal COP identified. He was in the state of respiratory fail- with an emphasis of pathologic findings. ure, and oxygen administration was performed. Cryptogenic organizing pneumonia

Figure 1. Cut surface of the left (A) and right (B) lungs. Both lungs are very hard and voluminous. Little air is present in the both lungs.

Then, he was treated by respirator, and by anti- Discussion biotics and steroids. Despite the steroid therapy, he showed a downhill course, and died one Clinically, the present case lacked no causative month after the first manifestation. The clinical agents. Microorganisms were not found. There diagnosis was acute pneumonia or ARDS. were no collagen-vascular diseases. No autoantibodies were found. Thus, the cause of the An autopsy was performed. The bilateral lungs pneumonia is idiopathic. Autopsy revealed dif- were voluminous (Figure 1A and 1B). The fuse organizing pneumonia involving the entire weight of lungs was 1050 g in the left lung lungs. Therefore, the present case is COP. (Figure 1A) and 1300 g in the right lung (Figure 1B). The both lungs were entirely affected The present case showed a rash course; the (Figure 1A and 1B). The lungs were hard, and interval between the first manifestation and little air was recognized. Microscopically, death was only one month. The patient did not almost all alveolar spaces contained Masson’s respond to steroid therapy. Such a very rash bodies (Figure 2A). Bronchiolitis obliterans was case of COP as the present case is very rare. In not recognized. The alveolar walls were not general, the prognosis of COP is relatively good, affected (Figure 2A). The Masson’s bodies and steroid is effective. However, the present showed collagenization with lymphocytic infil- case of COP was fatal. Fatal COP is very rare. tration (Figure 2B). Hyalinization of Masson’s bodies with little inflammatory infiltration was Autopsy cases of COP are very rare. The author frequently seen (Figure 2C). Cartilaginous could not find the references of autopsy cases metaplasia (Figure 2D) and ossification Figure( of COP in the English literature. Thus, the pres- 2E) of Masson’s bodies were seen in some ent autopsy case appears very important. At areas. No neutrophilic infiltration was recog- autopsy, the organizing pneumonia involved the nized. The pulmonary arteries were affected by entire lungs. Masson’s bodies showed variable fibrosis, and occasionally showed thrombosis stages of organization. Masson’s bodies with (Figure 2F). The pathological diagnosis was lymphocytic infiltration appear relatively recent COP. The heart weighted 500 g, and showed event. Masson’s bodies with hyalinization and right ventricular hypertrophy (cor pulmonale) little inflammatory infiltration appear old with the wall width of 0.7 cm (Figure 3). Other lesions. Of particular interest is that cartilagi- pathologic changes were pleural effusion (left, nous metaplasia and ossification were found in 800 ml: right, 1200 ml), acute liver congestion, places. The presence of these structures indi- prostatic hypertrophy, colon adenoma, and cates that the COP is very old one. Thus various hypercellular bone marrow. The cause of death stages of organizing pneumonia were found in was respiratory failure due to COP and pleural the present case. These findings suggest that effusion. the present patient had COP very previously.

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Figure 2. Microscopic findings. A: Low power view. The alveolar spaces are filled with numerous Masson’s bodies. The alveolar walls are not involved. Elastica van Gieson stain. X50. B: Masson’s bodies consisting of fibrious materials and lymphocytes. HE, x100. C: Masson’s bodies consisting of hyalinizaed collages. Elastica van Gieson stain, x100. D: Masson’s body showing cartilaginous metaplasia. HE, x100. E: Masson’s body showing ossification. HE, x100. F: Pulmonary artery containing thrombosis. HE, x50.

The presence of cor pulmonale in the present suggesting that long silent period of COP was case also suggests that the pulmonary lesions present in previous stages. The present case were old one. The cause of cor pulmonale did not show bronchiolitis obliterans, indicating appears due to pulmonary arteries involvement that the present is not BOOP but COP. In conclu- and pulmonary thrombosis. However, the pres- sion, the author reported an autopsy case of ent case clinically manifested very recently, fatal COP.

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References

[1] Cordier JF. Cryptogenic organizing pneumoni- tis: Bronchiolitis obliterans organizing pneumonia. Clin Chest Med 1993; 14: 677-692. [2] Cordier JF. Cryptogenic organizing pneumonia. Clin Chest Med 2004; 25: 727-738. [3] Alasaly K, Muller N, Ostrow DN, Champion P, FitzGerald JM. Cryptogenic organizing pneumonia: a report of 25 cases and a review of the literature. Medicine (Baltimore) 1995; 74: 201-211. [4] Barroso E, Hernandez L, Gil J, Garcia R, Aranda I, Romero S. Idiopathic organizing pneumonia. A relapsing disease: 19 years of experience in Figure 3. The heart. The heart weigh 500 g, and a hospital setting. Respiration 2007; 74: 624- shows right ventricular hypertrophy with the wall 631. width of 0.7 cm.

Conflict of interest statement

The author has no conflict of interest.

Address correspondence to: Dr. Tadashi Terada, Department of Pathology, Shizuoka City Shimizu Hospital, Miyakami 1231 Shimizu-Ku, Shizuoka 424-8636, Japan. Tel: +81-54-336-1111; Fax: +81- 54-334-1173; E-mail: [email protected]

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