Follicular bronchiolitis associated with rheumatoid arthritis.

Follicular bronchiolitis is a rare disorder, though it has been recognized as a pulmonary involvement of rheumatoid arthritis in recent years. A 57-year-old woman with rheumatoid arthritis was admitted to the hospital with persistent productive cough and breathlessness on exertion. An open lung biopsy was performed to establish a definite pathologic diagnosis of her disease, and she was diagnosed as having follicular bronchiolitis on the basis of the histopathological findings. It is essential to differentiate this disease from other bronchiolar or lymphoproliferative disorders of the lung.     

Follicular bronchiolitis preceding rheumatoid arthritis]

A 52-year-old woman visited our hospital because of a cough and stridor in 1994. She had a history of non-tuberculous mycobacteriosis and chronic paranasal sinusitis. Her chest CT scan showed scattered centrilobular nodular shadows and peribronchial thickening, and so we suspected recurrent non-tuberculous mycobacteriosis or diffuse panbronchiolitis. Clarithromycin treatment was initiated, but she soon ceased taking the drug. She visited our hospital again because of a severe cough three and half years later. A new chest CT scan showed increased abnormal shadows, and so we suspected worsening non-tuberculous mycobacteriosis or diffuse panbronchiolitis. Consequently, we commenced treatment with rifampicin, ethambutol hydrochloride as well as clarithromycin. One year later, rheumatoid arthritis was diagnosed because of a swollen proximal interphalangeal joint in both fourth fingers. Her respiratory symptoms were not relieved with clarithromycin or antituberculous drugs, so a thoracoscopic biopsy was performed for a more accurate diagnosis. Histological examination revealed follicular bronchiolitis.     

An autopsy case of obliterative bronchiolitis associated with Stevens-Johnson syndrome]

We reviewed an autopsied 27-year-old female with obliterative bronchiolitis associated with Stevens-Johnson syndrome. She had a history of Stevens-Johnson syndrome at age 10 years old and was treated with corticosteroids. Two months after the onset of dermatitis, the patient complained of dyspnea on exertion. The chest radiograph showed hyperinflation, and pulmonary function tests revealed obstructive impairment. The respiratory failure progressed due to respiratory tract infection and pneumothorax. She underwent thoracoscopic cyst surgery for right pneumothorax. Although the patient was clinically diagnosed as having obliterative bronchiolitis and received corticosteroids therapy and mechanical ventilation, she died of progressive respiratory failure 17 years after the onset of Stevens-Johnson syndrome. On autopsy, the macroscopic appearance of both lungs showed multiple white nodules in the centrilobular lesion corresponding to the obliteration of the small bronchioli. The microscopic appearance revealed constrictive bronchiolitis in the membranous bronchioli of both lungs associated with secondary bronchiectasis caused by superimposed infection.     

A case of bronchiolitis possibly associated with inhalation of hard metal]

A 36-year-old man, a worker exposed to tungsten and cobalt compounds, was admitted because of chest bilateral micronodular shadow with chronic cough and sputum. Chronic sinusitis, mild hypoxemia, obstructive respiratory impairment and chest radiological findings fulfilled the Japanese diagnostic criteria for diffuse panbronchiolitis, while atypical bronchoalveolar lavage fluid and pathological findings were seen. The surgical lung biopsy specimens showed patchy centrilobular inflammatory change with monocytic infiltrations and particulate deposition inside the area of bronchiolitis, but neither tungsten nor cobalt was found. Treatment with a macrolide antibiotic had no effect on the patient's symptoms, hypoxemia and lung function, suggesting bronchiolitis associated with inhalation of hard metal.     

A case of idiopathic bronchiolitis obliterans accompanied with pneumothorax and pulmonary atypical mycobacteriosis]

A 22-year-old man was urgently admitted for pneumothorax. He continued complaining of exertional dyspnea and dry cough after the pneumothorax healed. About three months later, an atypical pulmonary mycobacteriosis by mycobacterium kansasii was identified. Exertional dyspnea increased after chemotherapy was administered, and the patient was readmitted because of difficulty in daily life activities. Chest radiographs and CT scans showed bilateral pulmonary hyperinflation and a narrowed heart shadow. There was also marked combined ventilatory impairment, as identified by a respiratory function test. Furthermore, the histological findings of surgically removed lung tissue revealed accumulation of lymphocytes in the wall of a small bronchus. Idiopathic bronchiolitis obliterans was diagnosed from the clinical course and clinical findings. The patient is now being monitored and is awaiting lung transplantation.     

Familial occurrence of bronchiolitis obliterans associated with pulmonary aspergillosis

We report the familial occurrence of bronchiolitis obliterans (BO) associated with pulmonary aspergillosis. A mother and daughter admitted for dyspnea after taking Sauropus androgynus both had overfiltration of both lungs in chest X-ray and severe obstructive impairment in lung function tests. They were initially diagnosed with severe asthma and treated with high-dose prednisolone (0.5mg/kg/day) for 1 month to no effects. They did not show reversibility in lung function test. They were difinitively diagnosed with BO associated with Sauropus androgynus. Six months later, the mother's symptoms worsened with productive sputum and severe dyspnea, and her chest X-ray showed patty shadow in both upper lung fields. Precipitating antibodies to aspergillus antigen were positive and aspergillus fumigatus was detected from her sputum culture. She was diagnosed with aspergillosis and treatment with Micafungin (MCFG) and Itraconazole (ITCZ) was started. The daughter's symptoms also worsened and her chest tomography showed multiple cavities in both lung fields. Precipitating antibodies to aspergillus were positive. She was diagnosed with Invasive aspergillosis. She was treated unsuccessfully with MCFG and Amphotericin B (AMPH) and ITCZ. Few reports cover BO with aspergillus infection without organ impairment. In these 2 cases, treatment with steroids' or immune suppression of the lung with BO may a trigger aspergillus infection.     

A case of acute pulmonary embolism associated with squatting]

The patient was a 69-year-old man with a 3-year history of diabetes mellitus accompanied by cerebral infarction and arteriosclerosis obliterans of the lower extremities. After squatting to clean a car, the patient began to experience dyspnea. A blood gas analysis was performed upon admission, and the patient's PaCO2 and PaO2 levels were found to be low. Examination of a blood sample revealed elevated FDP, FDP-D dimer and platelet factor 4 levels. A perfusion lung scan showed multiple defects in both lungs, but no abnormal findings were detected on a ventilation scan. A pulmonary angiogram showed an interrupted blood flow in branches A9 + 10 of the left pulmonary artery. Accordingly, a pulmonary embolism was diagnosed. Squatting may have caused a partial obstruction of the venous return from the lower extremities by compression of the inguinal region and may have been a predisposing cause of the thrombosis. To our knowledge, such cases of pulmonary embolism triggered by the act of squatting are very rare.     

A case of pulmonary manifestation associated with myelodysplastic syndrome]

A 69-year-old woman with myelodysplastic syndrome (MDS) was admitted to our hospital because of recurrent fever and pulmonary infiltration shadows. On the seventh day of hospitalization, she had an attack of high fever and cough and laboratory tests revealed an elevated leukocyte count and elevated serum C-reactive protein. Chest radiographs showed infiltration shadows in the right middle and lower lung fields. Because a diagnosis of bacterial pneumonia was initially suggested, she was treated with antibiotics. However, the infiltration shadows on the chest radiograph had not improve, so bronchofiberscopy was performed. Analysis of fluid obtained by bronchoalveolar lavage (BAL) showed an increase in the total cell count, predominantly in lymphocytes and neutrophils. A transbronchial biopsy specimen showed infiltration of numerous neutrophils with necrosis under the bronchial epithelium, and edematous septa were infiltrated with numerous neutrophils and lymphocytes. BAL, blood, urine, bone marrow, and sputum cultures were all free of bacteria, mycobacteria and fungi. Interstitial infiltration by numbers of neutrophils associated with MDS was diagnosed and steroid treatment was performed.     

A case of primary pulmonary artery myxosarcoma associated with severe pulmonary hypertension]

A 50-year-old man presented with progressive dyspnea on exertion, but with no history of chest pain or syncope. Chronic pulmonary thromboembolism was suspected and he was referred to our hospital. On ausculation, a grade 3 systolic murmur was heard, that was loudest in the fifth intercostal space lateral to the right sternal border. Chest radiography showed mild cardiomegaly and ventilation-perfusion scan revealed absence of perfusion in the left lung and the upper field of the right lung. Contrast-enhanced helical CT showed large mural defects in both main pulmonary arteries, clearly delineated by contrast medium. The left pulmonary artery was nearly completely occluded, and eccentric defects were observed projecting into the lumen of the pulmonary trunk. A tumor originating in the pulmonary artery was suspected, but a definitive diagnosis of the mass could not be made with pulmonary angiography and magnetic resonance imaging. The mean pulmonary arterial pressure was 50 mmHg. Further radiologic examinations failed to reveal the source of the embolus or tumor. It was decided to attempt surgical excision under total cardiopulmonary bypass. At operation, a gelatinous, lustrous, yellowish mass was found partially occluding the right main pulmonary artery and completely occluding the left. The tumor adhered tightly to the intima of the vessel and was inoperable. The patient could not be weaned from percutaneous cardiopulmonary support and died 3 days after surgery. Histologic examination of the excised specimen revealed myxosarcoma.     

Follicular bronchiolitis in association with connective tissue diseases

Eleven patients with connective tissue diseases were found to have bronchiolar lesions associated with minimal or no alveolar septal thickening. Seven of these patients had rheumatoid arthritis, 3 had Sjögren’s syndrome, and 1 had ankylosing spondylitis. Radiographic studies showed an interstitial pattern in all patients. The patients were nonsmokers and received no treatment prior to biopsy. All lung biopsy specimens showed inflammatory bronchiolar lesions which consisted of bronchiolar and peribronchiolar lymphocytic infiltrates (follicular bronchiolitis). The bronchiolar lesion has been reported previously in patients with connective tissue diseases treated with D-penicillamine; whether it represents a component of the systemic disease or a lack of effect of the therapeutic agent had not been established. The present study shows that several connective tissue diseases can be associated with inflammatory bronchiolar lesions.     

A case of constrictive bronchiolitis]

A 31-year-old woman was admitted to our hospital because of a dry cough and progressive exertional dyspnea after her second delivery. She had almost normal laboratory data except for an elevated value for antibody for nucleic acid, and her chest radiograph was normal. However, pulmonary function tests revealed a mixed pattern of dysfunction, and chest CT revealed a mosaic perfusion pattern. The VATS lung biopsy demonstrated complete occlusion of the membranous bronchiole. These results led to a diagnosis of constrictive bronchiolitis (CB). The patient and her family rejected the option of lung transplantation, and selected immunosuppressive therapy (steroid pulse therapy and cyclophosphamide pulse therapy). Her condition improved temporarily, but her respiratory condition worsened progressively, and finally she died one and a half years after the appearance of the symptoms. Although steroid pulse therapy and cyclophosphamide pulse therapy failed to cure the CB, they did retard its progression.     

A case of juvenile pulmonary infarction associated with diet therapy]

A 31-year-old man experienced chest pain, fever, bloody sputum and cough after diet therapy. Chest radiography and chest CT showed infiltration in the right lower lung field and right pleural effusion. Pulmonary embolism and infarction was diagnosed using 99mTc-MAA perfusion scans and chest enhanced CT. The patient did not have a thrombotic disposition and deep vein thrombosis in the lower extremities. This case did not have an acute onset or dyspnea, and was not typical of pulmonary embolism. The diet therapy may have caused dehydration and acted as a predisposing cause of pulmonary embolism.     

A case of neurogenic pulmonary edema associated with epileptic seizure]

We report a case of neurogenic pulmonary edema associated with epileptic seizure. A 36-year-old woman had had several episodes of fainting and postictal respiratory failure, and since July 1998 had been admitted to a nearby hospital three times. On October 12, 1999, she was again admitted to a nearby hospital with the same symptom, and was transferred from that hospital to ours for evaluation of the recurrent respiratory disorder. Low-grade fever, mild leukocytosis, hypoxemia and bilateral diffuse opacities were observed as previously on chest radiography, and improved within several days without any specific therapy. The negative C reactive protein level, normal cardiac function and faintly bloody bronchoalveolar lavage fluid were also observed. There was no evidence of aspiration pneumonia, infectious disease, or underlying heart or lung disease. Electroencephalography showed spikes in accord with the left temporal lobe, and the cause of the patient's fainting was thought to be temporal lobe epilepsy. After all other causes had been excluded, this case was diagnosed as neurogenic pulmonary edema associated with epileptic seizure. Only about 40 cases of the postictal pulmonary edema have been reported since 1908, and the pathophysiologic mechanism of this condition is still unknown. Neurogenic pulmonary edema associated with epileptic seizure is rare, but the importance of awareness of this condition needs to be emphasized because it is suspected to be the cause of unexpected sudden death in epileptics. We should consider the disease as important in the differential diagnosis of acute respiratory failure associated with epilepsy.     

Case of chronic eosinophilic bronchiolitis associated with bronchial asthma]

A 62 year-old woman presented with diffuse, centriacinar nodular densities on chest radiography and CT, and an increase of peripheral blood eosinophils, four years after diagnosis of bronchial asthma. Diffuse panbronchiolitis was diagnosed, and was treated with erythromycin for a long period. One year later, she noticed exertional dyspnea, and her chest radiograph showed increased nodular densities. Lung biopsy under video-assisted thoracoscopy was performed, and revealed chronic bronchiolitis with eosinophilic infiltration, and focal, peribronchiolar eosinophilic infiltration in the alveolar septa and alveoli. She was treated with prednisolone, and her symptoms and nodular densities on chest radiography and CT were improved. We consider that the clinico-pathological findings of this case are consistent with those of chronic eosinophilic bronchiolitis, which has recently been reported in Japan. This case is different from previously reported ones in that eosinophilic bronchiolitis appeared in the course of bronchial asthma, suggesting the possibility that eosinophilic bronchiolitis may be accompanied with bronchial asthma or eosinophilic pneumonia.     

Familial occurrence of bronchiolitis obliterans associated with sauropus androgynus]

We experienced familial occurrence of bronchiolitis obliterans (BO) associated with Sauropus androgynus. The cases were a mother and daughter and both were admitted to our hospital because of dyspnea after taking Sauropus androgynus. Both cases had hyperinflation of both lungs in chest x-ray and lung function test showed severe obstructive impairment. At first, they were given a diagnosis of severe asthma and treated as such. However, neither their symptoms nor lung function improved. They did not show any reversibility on lung function tests. Although we did not perform histological examination of the lung, they were given a diagnosis of BO associated with Sauropus androgynus (SABO), because of the following reasons. Cases of SABO in Taiwan have already been demonstrated in the 1990's, and there were no other reasons to explain their severe airflow obstruction. Neither bronchodilators nor steroid treatment improved airflow obstruction. BO is rare and can mimic asthma and chronic obstructive pulmonary disease (COPD). We should inquire about the intake of food or medication in cases suspected BO.     

A case of eosinophilic bronchiolitis complicated with eosinophilic sinusitis]

A 42-year-old man was admitted to our hospital, complaining of dyspnea and cough for six months. Chest CT demonstrated thickening of the bronchial walls and some centrilobular nodules. From the laboratory data and the clinical course, he was first suspected to have bronchial asthma. However, it was necessary to rule out bronchiolitis due to other causes, because he had no previous asthma history and also because of the CT findings. We performed surgical lung biopsy to make a definite diagnosis. The pathological findings revealed eosinophilic bronchiolitis. While dyspnea and the eosinophilic nasal polyp improved by treatment with oral steroid therapy, the eosionophilic sinusitis and bronchiolitis relapsed after steroid tapering. Improvement and relapse of nasal and lower airway symptoms were synchronously observed. Eosinophilic bronchiolitis and eosionophilic sinusitis in this case may be considered to be a category of airway eosinophilic inflammation.     

A case of small cell lung cancer associated with pulmonary sarcoidosis]

A 63-year-old man with pulmonary sarcoidosis, diagnosed by mediastinal lymph node biopsy in 1977, was admitted in Feb. 1987 because of shortness of breath and cough. Chest X-ray showed bilateral hilar lymphadenopathy and a tumor shadow in the right lung field. Histological examination of specimens biopsied from the right lung revealed small cell carcinoma (S.C.C.). Bronchoalveolar lavage was performed to evaluate the disease activity of sarcoidosis, and the total number of cells and T-lymphocytes; the ratio of CD4+ cells to CD8+ cells was not increased. He was treated with combination chemotherapy, however, he died of respiratory failure after 7 months. An autopsy was performed, and the lesions were examined histologically. The sarcoid lesion in a lymph node obtained at autopsy was not active, in contrast to that obtained by mediastinal lymph node biopsy. Lung cancer and sarcoidosis are both common diseases, but their coexistence in the same patient is not common, and autopsied cases are rare. In this case, an autopsy was performed, and BAL had been performed prior to his death. The relationship between the BAL findings and the histology of sarcoidosis was examined. Based on the results of autopsy and BAL, the sarcoidosis was inactive prior to death, but had been histologically active 10 years previously. Therefore, this is a very interesting case, since we can examine the relationship between the two diseases, and the progression of each disease. This case also provides an interesting example of differentiation of sarcoidosis from S.C.C. Metastatic invasion of the hilar lymph nodes without bronchial stenosis and changes secondary to stenosis may often occur in patients with small cell lung cancer. Such metastatic invasion closely resembles the bilateral hilar lymphadenopathy of sarcoidosis; therefore, in some cases, it may be extremely difficult to differentiate the two diseases.