Cryptogenic Organizing Pneumonia - Idiopathic Bronchiolitis Obliterans Organizing Pneumonia

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A. Bekiæ al. Acta clinet Croat 2002; 43:281-287

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia Prefessional Paper

CRYPTOGENIC ORGANIZING PNEUMONIA – IDIOPATHIC BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA Anto Bekiæ1, Muharem Mehuliæ1, Diana Krmpotiæ1, Suzana Kukulj1, Marijan Goreèan1 and Šimun Križanac2 University Hospital for Pulmonary Diseases; 2Department of Pathology, School of Medicine, University of Zagreb, Zagreb, Croatia

1

SUMMARY – Cryptogenic organizing pneumonia is a rare pulmonary disease with characteristic clinical, radiologic and histologic features. The radiologic presentation, and ventilatory and respiratory lung functions reflect the presence of intra-alveolar buds of granulation tissue occurring within the alveoli and alveolar ducts but rarely occupying the bronchiolar lumen. Therefore, it has been accepted that the diagnosis of these characteristic but not specific presentations of cryptogenic organizing pneumonia requires histologic confirmation. The terms cryptogenic organizing pneumonia and idiopathic bronchiolitis obliterans organizing pneumonia are synonyms. There is also secondary organizing pneumonia casually related to various conditions. Presentation is made of two patients with different clinical manifestations of cryptogenic organizing pneumonia: one with low-grade chronic clinical course and migratory inflammatory lung infiltrates, and the other with severe acute clinical manifestations of the disease. In both patients with cryptogenic organizing pneumonia, corticosteroids showed high efficacy for both primary disease and relapses. Key words: Bronchiolitis obliterans organizing pneumonia – diagnosis; Bronchiolitis obliterans organizing pneumonia – therapy

Introduction Cryptogenic organizing pneumonia (COP) is defined as a rare inflammatory lung disease with characteristic clinicoradiologic and histopathologic manifestations. COP has been recognized as a distinct clinicopathologic entity by our clinicians1. No predisposing factors have been identified2. COP is also classified into interstitial inflammatory lung diseases of unknown cause2-4. The term COP is preferred to its synonyms such as primary or idiopathic bronchiolitis obliterans organizing pneumonia (BOOP)2,5. The characteristic radiologic pattern and lung function disorders reflect typical inflammatory lesions determined by the presence of buds of granulation tissue occurring within alveolar spaces and alveolar ducts but rarely occupying the bronchiolar lumen. Therefore, this characteristic clinicoradiologic entity of unknown cause with a particular pathologic hallmark of organizing pneumonia (OP) may be apCorrespondence to: Anto Bekiæ, M.D., M.S., University Hospital for Pulmonary Diseases, Jordanovac 104, HR-10000 Zagreb, Croatia E-mail: [email protected] Received April 26, 2004, accepted June 9, 2004

Acta clin Croat, Vol. 43, No. 3, 2004

propriately called COP to distinguish it from other types of bronchiolar diseases6-9. This pathoanatomical pattern of OP is not specific for COP but represents various mechanisms of the inflammatory reparatory process resulting from lung injury2,8,9. Although corticosteroids have been considered highly effective therapy for COP, the dosage and duration of treatment to recovery have not yet been clearly established6,10. Furthermore, relapses of COP are quite frequent after reducing or stopping the treatment with corticosteroids11. COP accounts for 50% of OPs, and the remaining 50% of OPs comprise of secondary organizing pneumonia (secondary BOOP), which is outlined herein4. It has been known that secondary OP may be found in association with certain drugs, infections, organ transplantation, following radiation therapy for breast cancer, connective tissue diseases, and many other causes6,7,12-21. There are no histologic, clinical or radiologic features to distinguish between COP and secondary OP. Corticosteroids are considered standard therapy for both COP and secondary OP. Therapeutic strategy for secondary OP depends on the nature of the underlying disorder. It has been generally accepted that the 281

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

first aim of treatment for secondary OP is removal of the offending agent or cause. The prognosis in secondary OP is usually worse than in COP.

Case Reports 1A

1C

1D

1B

Fig. 1A, 1B

Patient No. 1 A 72-year-old female presented with a one-month history of intermittent fever, cough and left-sided thoracic pain. She was a non-smoker. Chest radiography showed infiltrative opacity in the left inferior lung lobe (Fig. 1A). However, she did not improve with antibiotics administered for presumed infective pneumonia. Then she was admitted to the hospital. On admission, she appeared well, 282

1E Fig. 1C-E

afebrile, with crackles in the left subscapular lung region on auscultation. The erythrocyte sedimentation rate (ESR) rose to 51 m/h and C-reactive protein (CRP) was Acta clin Croat, Vol. 43, No. 3, 2004

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

increased (84.5 mm/L). The white blood cell (WBC) count was normal, with mild neutrophilia. There was mild arterial hypoxemia with normal spirometry and single-breath carbon monoxide diffusion capacity. Electrocardiography (ECG) and heart ultrasound (US) were normal. Bronchoscopic evaluation showed a normal tracheobronchial tree. Transbronchial lung biopsy specimen histology showed very thickened and fibrotic alveolar septa, some of them consisting of mononuclear infiltrations and accumulation of anthracotic pigment. Granulation tissue and foamy cells filled alveolar spaces. Bronchoalveolar lavage (BAL) predominantly showed lymphocytes (72%) and macrophages (18%), followed by eosinophils (6%) and granulocytes (4%). The patient remained clinically stable. Control chest radiography revealed infiltrates in the left lung base that regressed in size as compared with previous radiography findings, however, a new right lung infiltrative opacity occurred progressing in size despite antibiotic therapy administration (Fig. 1B). Therefore, the clinical and radiologic patterns as well as histologic findings pointed to COP as the most likely diagnosis. However, the patient refused the recommended corticosteroid therapy. Two months later, she was readmitted to the hospital for persistent cough, while clinically appearing unchanged. Radiographic control revealed partial dissolution of the inflammatory infiltrate in the right lung base and a new infiltrate in the left parahilar lung zone (Fig. 1C). The remainder of findings were not significantly changed. At this stage, the patient accepted corticosteroids and the treatment started with prednisolone, 40 mg. After 5 days of this therapy, her cough resolved. Radiographic controls initially showed partial dissolution of the lung infiltrates followed by complete dissolution in the further course of the disease. The dose of prednisolone was tapered to the maintenance dose. Corticosterid therapy was discontinued after 7 months. Relapses associated with symptom recurrence and radiographic finding of the left upper lung lobe infiltrate (Fig. 1D) corresponding to computed tomography (CT) finding of ground-glass attenuation (Fig. 1E) occurred two months of corticosteroid discontinuation. The patient received oral methylprednisolone, 24 mg, which resulted in rapid symptom disappearance and complete infiltrate dissolution on chest radiography. The dose of corticosteroid was tapered to the maintenance dose. Corticosteroid therapy was discontinued after 11 months. Patient No. 2 A 28-year-old female, a smoker, was referred to our hospital with a history of one-month malaise, generalized Acta clin Croat, Vol. 43, No. 3, 2004

2A 2B

Fig. 2A-B

fatigue, backpain, cough, body temperature up to 38 °C, and infiltrative opacities in both upper lobes on chest radiography. Prior to hospitalization, she was treated at outpatient department but did not respond to antibiotics and antituberculotics. Then she was admitted to the hospital in extremely poor condition. She was treated with alternative antibiotics and antimycotic fluconazole for oral candidiasis. On examination, she presented with bilateral bronchial breath sounds in the upper lung regions and audible left-sided crackles. Chest radiography showed consolidations containing air bronchograms in both upper lobes and apical segments of lower lobes, and possibly in lateral segment of the middle lobe (Fig. 2A). CT scan showed bilateral lung infiltrates with peripheral consolidated areas containing air bronchograms (Fig. 2B). Laboratory findings: ESR 124 mm/h; CRP 176 mg/L; WBC count 10.0x109 /L; and mild neutrophilia. On spirometry, she showed a severe restrictive ven283

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

tilatory defect and pronounced arterial hypoxemia. Bronchoscopy findings revealed normal appearance of the larynx and tracheobronchial tree, with bilateral purulent secretion within the lumen and traces of hemorrhage from the left sixth segemental bronchus. BAL was inadequate for histologic and immunocytologic examination. Sputum and BAL specimens referred for bacteriologic analysis were sterile, and cultures were negative for Mycobacterium tuberculosis. Histologically, transbronchial biopsy specimen of the sixth left segmental lung area showed alveolar spaces of normal size with some thickening of the alveolar septa and multiple intra-alveolar loose granulation tissues. There were some eosinophilic deposits within the alveoli and a minor accumulation of granulocytes predominated by eosinophils and some mononuclear cells in the alveolar walls. Based on the clinical presentation and these histologic findings, the diagnosis of COP was made and treatment with prednisolone, 60 mg per day, was initiated. Despite the dramatic onset of symptoms, this corticosteroid therapy led to rapid clinical response. Good clinical response including improved laboratory findings and partial dissolution of radiographic lung lesions was observed after a few days of corticosteroid therapy. Prednisolone was followed by methylprednisolone, 40 mg, with a rapid dose tapering to 32 mg, then slowly to the maintenance dose. After two months of corticosteroid therapy, the patient achieved full recovery. The follow-up imaging studies showed complete disappearance of lung lesions with mildly reduced total gas transfer and normal other laboratory findings. Corticosteroid therapy was discontinued after ten months. A relapse occurred two months of corticosteroid discontinuation. The patient presented with a mild clinical picture and identical but less numerous pulmonary lesions on chest radiography. Spirometry revealed a restrictive lung function defect of a higher degree and mild obstructive ventilatory defect. Total gas transfer was markedly reduced (46%), with arterial normoxemia. Corticosteroid therapy (32 mg methylprednisolone) with slow dose tapering was reintroduced with good clinical response. After six months of methylprednisolone (5 mg), the patient was clinically well and laboratory findings were normal except for a reduced total gas transfer (64%). It should be noted that the patient did not stop smoking.

Discussion Males and females are equally affected with COP, usually at the age between 40 and 60 years2,6. Our two patients were both of atypical age, one of them older and the other 284

one younger than the age most frequently associated with the disease occurrence. The typical onset of symptoms is subacute with fever, nonproductive cough, exertional dyspnea, anorexia and weight loss. The characteristic pulmonary infiltrates are typically present on chest radiography. Pleural effusion is rare2. Medical history frequently reveals unsuccessful treatment with antibiotics administered for presumed infective pneumonia. On physical examination, inspiratory crackles may be present. The clinical presentation of COP may be typical and focal in about 80% and 10% of COP patients, respectively5. The focal variant of COP may present radiographically as a solitary node or multiple nodular lesions, and clinically often with pleural pain. The diagnosis of COP is usually made histologically2,22. The fibrotic progressive and nonprogressive forms are rare, and fulminant form is extremely rare5,23. Clinically, the fulminant variant of COP may be similar to acute interstitial pneumonia and adult respiratory distress syndrome (ARDS) followed by acute respiratory failure over a few days24. Both of our patients had fever, cough and pulmonary infiltrates that did not resolve with antibiotic therapy. Patient No. 1 was in good condition and presented with a slowly progressive clinical course. Patient No. 2 was in extremely poor condition with a rapidly progressive form of the disease. On physical examination, patient No. 1 typically presented with audible crackles over the radiologically detected lung infiltrate, whereas patient No. 2 presented with bronchial breath sounds such as those heard in bacterial pneumonia. Although laboratory findings are not specific for the diagnosis of COP, accelerated ESR and increased CRP values are commonly found. Moderate leukocytosis with variable neutrophilia may be present in 15%-35% of patients6. Both of our patients had accelerated ESR, increased CRP values and mild neutrophilia, however, without any major increase in total leukocyte count. Radiographic findings may show bilateral inhomogeneous alveolar consolidations in peripheral lung areas and lower lung lobes. Other radiographic patterns include reticular interstitial opacities or fibrotic lesions. The size of consolidations varies from a few centimeters to a whole lobe. Rare unilateral and migratory infiltrates may be found in about 25% of COP patients2,20,25. The progressive form of COP typically shows slow enlargement of infiltrates, which may also occur at another localization. Central colliquations are rarely found, and may be seen in the focal form of COP2. Pleural effusion is rarely observed. It has been generally accepted that the characteristic CT findings in association with typical clinical presentation suggest the diagnosis of Acta clin Croat, Vol. 43, No. 3, 2004

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

COP2,26. Ground-glass attenuation and inhomogeneous alveolar consolidations are most often reported (90%-100% and 83%-91%, respectively)2. The consolidations typically have a triangular appearance with the basis on the pleural side and the apex turning on the hilus2. The consolidations usually contain air bronchograms such as those seen in pneumonia17. CT scans may also show linear opacities and rarely reticulonodular opacities2. The characteristic radiographic presentations of COP were detected in both of our patients: patient No. 1 presented with migratory pulmonary infiltrates, and patient No. 2 with extensive progressive infiltrates. As expected, no characteristic bronchoscopic findings related to COP were found in either of our patients. It has been usually reported that cytologically BAL may show a “mixed pattern” with obligatorily increased lymphocyte count (20%-40%) and mild granulocytosis (

Deskripsi

A. Bekiæ al. Acta clinet Croat 2002; 43:281-287

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia Prefessional Paper

CRYPTOGENIC ORGANIZING PNEUMONIA – IDIOPATHIC BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA Anto Bekiæ1, Muharem Mehuliæ1, Diana Krmpotiæ1, Suzana Kukulj1, Marijan Goreèan1 and Šimun Križanac2 University Hospital for Pulmonary Diseases; 2Department of Pathology, School of Medicine, University of Zagreb, Zagreb, Croatia

1

SUMMARY – Cryptogenic organizing pneumonia is a rare pulmonary disease with characteristic clinical, radiologic and histologic features. The radiologic presentation, and ventilatory and respiratory lung functions reflect the presence of intra-alveolar buds of granulation tissue occurring within the alveoli and alveolar ducts but rarely occupying the bronchiolar lumen. Therefore, it has been accepted that the diagnosis of these characteristic but not specific presentations of cryptogenic organizing pneumonia requires histologic confirmation. The terms cryptogenic organizing pneumonia and idiopathic bronchiolitis obliterans organizing pneumonia are synonyms. There is also secondary organizing pneumonia casually related to various conditions. Presentation is made of two patients with different clinical manifestations of cryptogenic organizing pneumonia: one with low-grade chronic clinical course and migratory inflammatory lung infiltrates, and the other with severe acute clinical manifestations of the disease. In both patients with cryptogenic organizing pneumonia, corticosteroids showed high efficacy for both primary disease and relapses. Key words: Bronchiolitis obliterans organizing pneumonia – diagnosis; Bronchiolitis obliterans organizing pneumonia – therapy

Introduction Cryptogenic organizing pneumonia (COP) is defined as a rare inflammatory lung disease with characteristic clinicoradiologic and histopathologic manifestations. COP has been recognized as a distinct clinicopathologic entity by our clinicians1. No predisposing factors have been identified2. COP is also classified into interstitial inflammatory lung diseases of unknown cause2-4. The term COP is preferred to its synonyms such as primary or idiopathic bronchiolitis obliterans organizing pneumonia (BOOP)2,5. The characteristic radiologic pattern and lung function disorders reflect typical inflammatory lesions determined by the presence of buds of granulation tissue occurring within alveolar spaces and alveolar ducts but rarely occupying the bronchiolar lumen. Therefore, this characteristic clinicoradiologic entity of unknown cause with a particular pathologic hallmark of organizing pneumonia (OP) may be apCorrespondence to: Anto Bekiæ, M.D., M.S., University Hospital for Pulmonary Diseases, Jordanovac 104, HR-10000 Zagreb, Croatia E-mail: [email protected] Received April 26, 2004, accepted June 9, 2004

Acta clin Croat, Vol. 43, No. 3, 2004

propriately called COP to distinguish it from other types of bronchiolar diseases6-9. This pathoanatomical pattern of OP is not specific for COP but represents various mechanisms of the inflammatory reparatory process resulting from lung injury2,8,9. Although corticosteroids have been considered highly effective therapy for COP, the dosage and duration of treatment to recovery have not yet been clearly established6,10. Furthermore, relapses of COP are quite frequent after reducing or stopping the treatment with corticosteroids11. COP accounts for 50% of OPs, and the remaining 50% of OPs comprise of secondary organizing pneumonia (secondary BOOP), which is outlined herein4. It has been known that secondary OP may be found in association with certain drugs, infections, organ transplantation, following radiation therapy for breast cancer, connective tissue diseases, and many other causes6,7,12-21. There are no histologic, clinical or radiologic features to distinguish between COP and secondary OP. Corticosteroids are considered standard therapy for both COP and secondary OP. Therapeutic strategy for secondary OP depends on the nature of the underlying disorder. It has been generally accepted that the 281

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

first aim of treatment for secondary OP is removal of the offending agent or cause. The prognosis in secondary OP is usually worse than in COP.

Case Reports 1A

1C

1D

1B

Fig. 1A, 1B

Patient No. 1 A 72-year-old female presented with a one-month history of intermittent fever, cough and left-sided thoracic pain. She was a non-smoker. Chest radiography showed infiltrative opacity in the left inferior lung lobe (Fig. 1A). However, she did not improve with antibiotics administered for presumed infective pneumonia. Then she was admitted to the hospital. On admission, she appeared well, 282

1E Fig. 1C-E

afebrile, with crackles in the left subscapular lung region on auscultation. The erythrocyte sedimentation rate (ESR) rose to 51 m/h and C-reactive protein (CRP) was Acta clin Croat, Vol. 43, No. 3, 2004

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

increased (84.5 mm/L). The white blood cell (WBC) count was normal, with mild neutrophilia. There was mild arterial hypoxemia with normal spirometry and single-breath carbon monoxide diffusion capacity. Electrocardiography (ECG) and heart ultrasound (US) were normal. Bronchoscopic evaluation showed a normal tracheobronchial tree. Transbronchial lung biopsy specimen histology showed very thickened and fibrotic alveolar septa, some of them consisting of mononuclear infiltrations and accumulation of anthracotic pigment. Granulation tissue and foamy cells filled alveolar spaces. Bronchoalveolar lavage (BAL) predominantly showed lymphocytes (72%) and macrophages (18%), followed by eosinophils (6%) and granulocytes (4%). The patient remained clinically stable. Control chest radiography revealed infiltrates in the left lung base that regressed in size as compared with previous radiography findings, however, a new right lung infiltrative opacity occurred progressing in size despite antibiotic therapy administration (Fig. 1B). Therefore, the clinical and radiologic patterns as well as histologic findings pointed to COP as the most likely diagnosis. However, the patient refused the recommended corticosteroid therapy. Two months later, she was readmitted to the hospital for persistent cough, while clinically appearing unchanged. Radiographic control revealed partial dissolution of the inflammatory infiltrate in the right lung base and a new infiltrate in the left parahilar lung zone (Fig. 1C). The remainder of findings were not significantly changed. At this stage, the patient accepted corticosteroids and the treatment started with prednisolone, 40 mg. After 5 days of this therapy, her cough resolved. Radiographic controls initially showed partial dissolution of the lung infiltrates followed by complete dissolution in the further course of the disease. The dose of prednisolone was tapered to the maintenance dose. Corticosterid therapy was discontinued after 7 months. Relapses associated with symptom recurrence and radiographic finding of the left upper lung lobe infiltrate (Fig. 1D) corresponding to computed tomography (CT) finding of ground-glass attenuation (Fig. 1E) occurred two months of corticosteroid discontinuation. The patient received oral methylprednisolone, 24 mg, which resulted in rapid symptom disappearance and complete infiltrate dissolution on chest radiography. The dose of corticosteroid was tapered to the maintenance dose. Corticosteroid therapy was discontinued after 11 months. Patient No. 2 A 28-year-old female, a smoker, was referred to our hospital with a history of one-month malaise, generalized Acta clin Croat, Vol. 43, No. 3, 2004

2A 2B

Fig. 2A-B

fatigue, backpain, cough, body temperature up to 38 °C, and infiltrative opacities in both upper lobes on chest radiography. Prior to hospitalization, she was treated at outpatient department but did not respond to antibiotics and antituberculotics. Then she was admitted to the hospital in extremely poor condition. She was treated with alternative antibiotics and antimycotic fluconazole for oral candidiasis. On examination, she presented with bilateral bronchial breath sounds in the upper lung regions and audible left-sided crackles. Chest radiography showed consolidations containing air bronchograms in both upper lobes and apical segments of lower lobes, and possibly in lateral segment of the middle lobe (Fig. 2A). CT scan showed bilateral lung infiltrates with peripheral consolidated areas containing air bronchograms (Fig. 2B). Laboratory findings: ESR 124 mm/h; CRP 176 mg/L; WBC count 10.0x109 /L; and mild neutrophilia. On spirometry, she showed a severe restrictive ven283

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

tilatory defect and pronounced arterial hypoxemia. Bronchoscopy findings revealed normal appearance of the larynx and tracheobronchial tree, with bilateral purulent secretion within the lumen and traces of hemorrhage from the left sixth segemental bronchus. BAL was inadequate for histologic and immunocytologic examination. Sputum and BAL specimens referred for bacteriologic analysis were sterile, and cultures were negative for Mycobacterium tuberculosis. Histologically, transbronchial biopsy specimen of the sixth left segmental lung area showed alveolar spaces of normal size with some thickening of the alveolar septa and multiple intra-alveolar loose granulation tissues. There were some eosinophilic deposits within the alveoli and a minor accumulation of granulocytes predominated by eosinophils and some mononuclear cells in the alveolar walls. Based on the clinical presentation and these histologic findings, the diagnosis of COP was made and treatment with prednisolone, 60 mg per day, was initiated. Despite the dramatic onset of symptoms, this corticosteroid therapy led to rapid clinical response. Good clinical response including improved laboratory findings and partial dissolution of radiographic lung lesions was observed after a few days of corticosteroid therapy. Prednisolone was followed by methylprednisolone, 40 mg, with a rapid dose tapering to 32 mg, then slowly to the maintenance dose. After two months of corticosteroid therapy, the patient achieved full recovery. The follow-up imaging studies showed complete disappearance of lung lesions with mildly reduced total gas transfer and normal other laboratory findings. Corticosteroid therapy was discontinued after ten months. A relapse occurred two months of corticosteroid discontinuation. The patient presented with a mild clinical picture and identical but less numerous pulmonary lesions on chest radiography. Spirometry revealed a restrictive lung function defect of a higher degree and mild obstructive ventilatory defect. Total gas transfer was markedly reduced (46%), with arterial normoxemia. Corticosteroid therapy (32 mg methylprednisolone) with slow dose tapering was reintroduced with good clinical response. After six months of methylprednisolone (5 mg), the patient was clinically well and laboratory findings were normal except for a reduced total gas transfer (64%). It should be noted that the patient did not stop smoking.

Discussion Males and females are equally affected with COP, usually at the age between 40 and 60 years2,6. Our two patients were both of atypical age, one of them older and the other 284

one younger than the age most frequently associated with the disease occurrence. The typical onset of symptoms is subacute with fever, nonproductive cough, exertional dyspnea, anorexia and weight loss. The characteristic pulmonary infiltrates are typically present on chest radiography. Pleural effusion is rare2. Medical history frequently reveals unsuccessful treatment with antibiotics administered for presumed infective pneumonia. On physical examination, inspiratory crackles may be present. The clinical presentation of COP may be typical and focal in about 80% and 10% of COP patients, respectively5. The focal variant of COP may present radiographically as a solitary node or multiple nodular lesions, and clinically often with pleural pain. The diagnosis of COP is usually made histologically2,22. The fibrotic progressive and nonprogressive forms are rare, and fulminant form is extremely rare5,23. Clinically, the fulminant variant of COP may be similar to acute interstitial pneumonia and adult respiratory distress syndrome (ARDS) followed by acute respiratory failure over a few days24. Both of our patients had fever, cough and pulmonary infiltrates that did not resolve with antibiotic therapy. Patient No. 1 was in good condition and presented with a slowly progressive clinical course. Patient No. 2 was in extremely poor condition with a rapidly progressive form of the disease. On physical examination, patient No. 1 typically presented with audible crackles over the radiologically detected lung infiltrate, whereas patient No. 2 presented with bronchial breath sounds such as those heard in bacterial pneumonia. Although laboratory findings are not specific for the diagnosis of COP, accelerated ESR and increased CRP values are commonly found. Moderate leukocytosis with variable neutrophilia may be present in 15%-35% of patients6. Both of our patients had accelerated ESR, increased CRP values and mild neutrophilia, however, without any major increase in total leukocyte count. Radiographic findings may show bilateral inhomogeneous alveolar consolidations in peripheral lung areas and lower lung lobes. Other radiographic patterns include reticular interstitial opacities or fibrotic lesions. The size of consolidations varies from a few centimeters to a whole lobe. Rare unilateral and migratory infiltrates may be found in about 25% of COP patients2,20,25. The progressive form of COP typically shows slow enlargement of infiltrates, which may also occur at another localization. Central colliquations are rarely found, and may be seen in the focal form of COP2. Pleural effusion is rarely observed. It has been generally accepted that the characteristic CT findings in association with typical clinical presentation suggest the diagnosis of Acta clin Croat, Vol. 43, No. 3, 2004

A. Bekiæ et al.

Cryptogenic organizing pneumonia – idiopathic bronchiolitis obliterans organizing pneumonia

COP2,26. Ground-glass attenuation and inhomogeneous alveolar consolidations are most often reported (90%-100% and 83%-91%, respectively)2. The consolidations typically have a triangular appearance with the basis on the pleural side and the apex turning on the hilus2. The consolidations usually contain air bronchograms such as those seen in pneumonia17. CT scans may also show linear opacities and rarely reticulonodular opacities2. The characteristic radiographic presentations of COP were detected in both of our patients: patient No. 1 presented with migratory pulmonary infiltrates, and patient No. 2 with extensive progressive infiltrates. As expected, no characteristic bronchoscopic findings related to COP were found in either of our patients. It has been usually reported that cytologically BAL may show a “mixed pattern” with obligatorily increased lymphocyte count (20%-40%) and mild granulocytosis (
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