A case of the limited from of Wegener's granulomatosis without c-ANCA]

A 46-year-old woman was admitted to our hospital because of fever, cough and headache in December 2001. Although she had been treated for nasal obstruction and epistaxis by an otorhinolaryngologist in our hospital since 1996, no accurate diagnosis had been made despite repeated biopsies of the nasal mucosa. A chest CT taken in 1999 showed ground-glass opacities in both upper lobes. On admission, chest radiography and CT showed mass shadows without cavitation, corresponding to the lesions causing the ground-glass opacities. In addition, paranasal sinus MRI showed a deformity of the nasal septum accompanied by a space-occupying lesion, suggesting Wegener's granulomatosis. However, the cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA) test was negative. To achieve a definitive diagnosis, we performed an open lung biopsy. The specimen, obtained from the right upper lobe, showed the typical findings of a Wegener's granulomatosis including necrotizing vasculitis. Oral prednisolone treatment initiated at 20 mg daily, combined with oral cyclophosphamide at 50 mg daily markedly improved not only the clinical symptoms, but also the mass shadows in the left upper lobe. Patients with the limited form of Wegener's granulomatosis are occasionally seronegative and respond well to therapy. However, the natural course and the changes in chest radiographs are not understood well in such cases. In this paper, we report a case of the limited form of Wegener's granulomatosis that progressed slowly over a period of 6 years.     

A case of limited form of Wegener's granulomatosis showing repeated occurrence and disappearance of nodules in chest X ray with no medication]

A 69-year-old woman was admitted to because of bloody sputum. Chest CT showed some nodules. The patient was seronegative for PR3-ANCA. One month after the admission, these shadows vanished with no medication. She was asymptomatic for one and a half years, and then she complained of cough again. Multiple nodules were seen on chest CT again. She was admitted, and surgical biopsy by video-assisted thoracoscopic surgery was performed. She was diagnosed to have a limited form of Wegener's granulomatosis. Multiple shadows vanished without medication, again. Administration of sulfamethoxazole-trimethoprim was started and there has been no relapse.     

A case of Wegener's granulomatosis without PR3-ANCA at relapse]

A 49-year-old man was admitted to our hospital with fever. His chest radiograph showed some nodules in the right upper and lower lung fields. The cytoplasmic-antineutrophil cytoplasmic antibody test was positive, and histopathologic biopsy of a small nasal polyps yielded a diagnosis of Wegener's granulomatosis. He was started on prednisolone and cyclophosphamide. The findings on his chest radiograph and his symptoms improved rapidly, and we stopped these drugs after one year. Two years after cessation of treatment, his chest radiograph showed two nodules with cavities. Relapse of Wegener's granulomatosis was diagnosed. The proteinase 3-antineutrophil cytoplasmic antibody test was negative. He was started on prednisolone and cyclophosphamide, and the findings on his chest radiograph improved rapidly. Chest radiographs are useful for follow-up observation of patients with Wegener's granulomatosis after treatment.     

A case of plasma cell granuloma with good response to steroid therapy

A 46-year-old man was admitted with complaints of fever, cough and anterior chest pain. Chest X-ray examination disclosed an infiltrative shadow in the right upper lung field and chest CT scan strongly suggested invasion of mediastinum and anterior chest wall. Therefore, steroid therapy was selected because biopsy specimen showed proliferation of fibroblasts and mononuclear cells mainly containing plasma cells with no evidence of malignancy. After steroid therapy, chest X-ray examination showed that the infiltrative shadow was greatly reduced. However, since infiltrative shadow was increased again at 3 years after the initiation of steroid therapy, right upper lobectomy was performed. Histopathological analysis of resected tumor demonstrated proliferation of mature plasma cells and fibroblasts in conjunction with minor infiltration of neutrophils and lymphocytes. At 18 months after operation, new shadows appeared in right S8 and S10 which were shown to have almost the same histological findings as the resected tumor. Steroid therapy was restarted, and then the size of shadows decreased. It is conceivable that steroid therapy is an effective therapy for pulmonary plasma cell granuloma, especially multifocal and relapsing cases.     

A case of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment]

A 23-year-old man was admitted to our hospital for a complete medical evaluation of abnormal pulmonary shadows found on a chest radiograph during his annual check-up. Chest radiography and chest CT showed a diffuse spread of micronodules in both lung fields and mediastinal lymphadenopathy. A transbronchial lung biopsy demonstrated evidence of noncaseating epithelioid cell granuloma with multinucleated giant cells, and a diagnosis of sarcoidosis was made. The pulmonary shadows improved without therapy. Twenty months later, the patient was readmitted to the hospital because of chest pain and dyspnea. Pneumothorax appeared on the right in a chest radiograph, but subsided after drainage therapy, and two weeks later, a right side pleural effusion was seen. We determined that the pleura was involved in the sarcoidosis, and the patient was treated with oral prednisolone 20 mg daily. The pleural effusion gradually subsided. This is the first reported case in Japan of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment.     

A case of pneumonitis due to sho-saiko-to

We reported a case of pneumonitis due to Sho-saiko-to. A 71-year-old woman was admitted to our hospital because of pneumonia. She complained of dry cough, pyrexia and severe dyspnea. Fine crepitation was heard on physical examination of the chest and a chest X-ray film revealed diffuse reticulo-nodular shadow in both lung fields. We suspected summer-type hypersensitivity pneumonitis and followed her up, however her condition deteriorated. Under a suspicion of drug-induced pneumonitis, all drugs were stopped and she was given prednisolone. Consequently her complaints, laboratory data and chest X-ray findings markedly improved. Microscopic examination of a transbronchial lung biopsy specimens showed interstitial pneumonitis. The results of a lymphocyte stimulation test were positive for sho-saiko-to. She gave informed consent after receiving an explanation of the challenge test. She was tested with 2.5 g sho-saiko-to twice and developed high fever and dyspnea with hypoxia, while the chest X-ray film also revealed diffuse infiltrative shadows similar that on admission. Based on these findings, we diagnosed this case as pneumonitis due to sho-saiko-to. To our knowledge, there has been no previous case of pulmonary hypersensitivity due to sho-saiko-to reported in the world.     

A case of Wegener's granulomatosis presenting as severe acute respiratory failure]

A 19-year-old man was admitted to another hospital. Pulmonary suppuration was diagnosed and was treated with antibiotics. However, he developed acute respiratory failure, which required intubation and ventilation with 100% oxygen. After treatment with nitric oxide inhalation and corticosteroid pulse therapy, the patient's condition stabilized and he gradually regained a satisfactory pulmonary function. He was discharged about 3 months after admission with a pulmonary function close to normal. Approximately 1 month later, the patient was admitted to our hospital because of a 2-week history of fever and chest and ocular pain. A chest radiograph obtained upon admission showed a nodule with a cavity in the upper lobe of the right lung. Pulmonary suppuration was again suspected, and antibiotics were given. The fever persisted and chest radiograph on hospital day 19 showed marked extension of the nodules. At that time, the patient complained of nasal obstruction and hoarseness and his sclera showed intense congestion, indicating episcleritis. Wegener's granulomatosis was diagnosed on the basis of the clinical picture, PR3-ANCA titer (63 EU) and nasal biopsy findings. After treatment with prednisolone and cyclophosphamide, his condition stabilized, and he recovered gradually. However, his condition remains poor despite continued therapy. This is an extremely rare case of Wegener's granulomatosis presenting as severe acute respiratory failure.     

A case of bronchiolitis obliterans organizing pneumonia in a patient with rheumatoid arthritis

A 54-year-old man with rheumatoid arthritis visited his general practitioner because of fever and cough. Chest X-ray showed an infiltrative shadow in the right lower field. Antibiotic treatment was not effective, and the specimens obtained by transbronchial lung biopsy was not diagnostic. The patient was transferred to our hospital for further examination and treatment. Previously he had been treated with prednisolone in the knee joint for rheumatoid arthritis. Open lung biopsy was performed. The specimen obtained showed bronchiolitis obliterans organizing pneumonia (BOOP) histologically. The patient recuperated and the chest X-ray shadow decreased with no therapy except the previous treatment with prednisolone.     

A case of isoniazid (INH)-induced pneumonitis]

A 58-year-old man was referred for the evaluation of a lung nodule on chest X-ray. On admission, chest X-ray showed a solitary nodule with cavitation in the left lung field. Histological examination revealed epithelioid cell granulomas and the diagnosis of pulmonary tuberculosis was made. He was treated with INH, ethambutol (EB), and rifampicin (RFP). On the 16th day of treatment, he developed dry cough and high fever. On the 20th day, dyspnea developed and PaO2 was decreased to 38.2 Torr. Chest X-ray showed new widespread infiltrates in both lung fields and bilateral pleural effusions. The size of the cavitary lesion was decreased. Transbronchial biopsy specimen showed slight interstitial thickening, lymphocyte infiltration, and multiple granulomas. Drug lymphocyte stimulation test was positive only with INH (230%). INH-induced pneumonitis was highly suspected. All drugs was discontinued and hydrocortisone 2400 mg daily was started. He soon became afebrile, and dyspnea and dry cough resolved. Chest X-ray film showed resolution of infiltrative shadows. He was subsequently successfully treated with streptomycin, EB, and RFP without any adverse effects. To our knowledge, this is the sixth reported case of INH-induced pneumonitis.     

A relapsing case of Wegener's granulomatosis presenting as an endobronchial mass

Wegener's granulomatosis (WG) relapse is frequent. Although lung involvement occurs in 85% of patients, endobronchial presentation of the disease is uncommon. We reported a relapsing case of WG presenting as an endobronchial mass. A 56- year-old man present ed with recurrence of WG following 14 months of cyclophosphamide and prednisolone therapy and 36 months of complete remission. At his first presentation, he was diagnosed as having WG with involvement of kidney, lung, upper airways, skin, joints and eyes. His chest X-ray showed bilateral patchy consolidation. Cytoplasmic-anti-neutrophil cytoplasmic antibodies (c-ANCA) was also present in high titres. c-ANCA was negative after therapy. At the time of relapse, he presented with nasal symptoms and hemoptysis. His chest X-ray showed right paracardiac opacity. Fiberoptic bronchoscopy revealed a mass lesion subtotally obstructing the proximity of right lower lobe. He has been given prednisolone in tapering doses and cyclophosphamide for 10 months.     

Idiopathic bronchiolitis obliterans organizing pneumonia complicated by transitory cystic lesion in the healing stage]

A 21-year-old man was admitted to our hospital because chest X-ray films disclosed infiltrative shadows indicative of Mycoplasma pneumonia. He experienced fever and dry cough for 2 weeks. Chest X-ray findings showed ground-glass shadows in the lower fields of both lungs. The patient was not responsive to antibiotic therapy (PAPM/BP 1 g/day and CLDM 1,200 mg/day); dyspnea advanced rapidly and spikes of fever persisted. On hospital day 5, chest computed tomographic (CT) films disclosed interstitial shadows in all lower lung fields with dense infiltration. A transbronchial lung biopsy (TBLB) was performed on day 7, and TBLB specimens demonstrated infiltration of mononuclear cells in alveolar septa and organizing exudate in alveolar ducts with polypoid granulation tissue. Bronchoalveolar lavage fluid findings revealed an increase in the total cell count and the percentage of lymphocytes. The CD 4/CD 8 ratio was normal. The findings of other laboratory tests ruled out drug-induced lung disease, infectious disease, and collagen disease. Idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP) was diagnosed. Corticosteroid therapy (methyl prednisolone: 500 mg/day) was started. After 2 weeks of treatment (prednisolone: 30 mg/day), the dyspnea and fever disappeared. Chest CT films showed that the interstitial shadows had largely resolved, but that a large cystic lesion had formed rapidly in the right lower lung field (right S 6). To the best of our knowledge, no cases of BOOP complicated by cystic lesions in the healing stage have been reported to date. We speculated that polypoid granulation in a bronchiole had given rise to a check-valve mechanism. After 2 months of treatment (prednisolone: 15 mg/day), the cystic lesion disappeared. We concluded that the bronchiolar lesion of polypoid granulation had resolved in response to therapy, thus facilitating air-way communication and the release of air from the cyst.     

A case of Sweet's syndrome with a variety of chest radiological findings]

We report a rare case of Sweet's syndrome (acute febrile neutrophilic dermatosis) with a variety of chest radiological findings. A 73-year-old man, who had been treated with corticosteroid for Sweet's syndrome for 2 years, was admitted to our hospital because of pyrexia with a infiltrative chest shadow. Chest CT scans showed consolidation and ground-glass opacities with air-bronchogram in the right lower lobe. Treatment with antibiotics seemed to be effective but there was no improvement of chest shadows. Simultaneously with his pyrexia, diffuse centrilobular-micronodular shadows and a mass-like shadow appeared on chest CT after 2 months and after the next 2 months, respectively. Bronchoalveolar lavage fluid contained increased neutrophils but not any infectious pathogen. Transbronchial lung biopsy specimens revealed chronic interstitial infiltrate with alveolar wall thickening and neutrophil accumulation in the airspace. A diagnosis of pulmonary involvement in a patient with Sweet's syndrome was finally made and he was successfully treated with corticosteroid.     

A case of necrotizing sarcoid granulomatosis diagnosed by open lung biopsy

A 32-year-old female complained of productive cough and bloody sputum. Infiltrative shadows and cavitary lesions with thick and irregular wall in the bilateral lung fields and the swelling of mediastinal lymph nodes were pointed out on the chest radiography. Physical examination revealed no abnormal findings. ESR and the level of the serum angiotensin converting enzyme were slightly elevated. In the BAL (broncho-alveolar lavage) fluid, lymphocytes and neutrophils increased, and the OKT4/OKT8 ratio of the lymphocytes was 0.81. Open lung biopsy revealed numerous sarcoid granulomas with granulomatous vasculitis in the cavity wall, surrounding infiltrative lesions and hilar lymph nodes. After the administration of prednisolone, the infiltrative shadows and the cavitary lesions showed marked improvement. It was concluded that open lung biopsy is necessary for the diagnosis of NSG because the differential diagnosis between NSG and limited form of Wegener's granulomatosis is extremely difficult from such a small lung specimen as that obtained by trans-bronchial lung biopsy.     

A case of adult onset idiopathic pulmonary hemosiderosis markedly improved by steroid therapy]

A case of adult onset idiopathic pulmonary haemosiderosis (IPH) was reported. A 53-year-old man was admitted to our hospital because of repeated bloody sputum on June 2, 2006. Chest radiograph on admission disclosed diffuse infiltrative shadows in both lung fields, and one month later these shadows became more marked. The chest CT on July 5, 2006 showed patchy areas of ground-glass opacity and consolidation, exhibiting a distinctly peripheral distribution. Bronchoscopic findings revealed oozing bleeding from the orifice of B5 in the right lung and B9 in the left lung. We employed video-assisted thoracoscopic surgery for lung biopsy and he as primary IPH was diagnosed clinicopathologically. His symptoms and radiographic findings were markedly improved after steroid therapy, followed by no signs of recurrence. It may be important to establish a definitive diagnosis early, even in IPH, using VATS, for further effective therapy.     

A case of Wegener's granulomatosis complicated by hypopituitarism]

A 71-year-old male complaining of chest pain was admitted to our hospital. A single cavitary mass shadow was observed on chest X-ray films. Urinalysis revealed microscopic hematuria. CT examination demonstrated a tumorous shadow in the maxillary sinus. The diagnosis of Wegener's granulomatosis was histologically established by biopsy specimens from the nasal mucosa which showed necrotizing vasculitis and granuloma with fibrinoid degeneration. He was treated with combination therapy of prednisolone and cyclophosphamide. The abnormal shadows on chest X-ray and in the maxillary sinus on CT improved rapidly, but the patient developed progressive weight loss and complained of cold intolerance, weakness and dysphagia. Serum T3, T4 and TSH were found to be reduced. Anterior pituitary function tests showed reduction of TSH, GH and ACTH responses, which was probably due to irreversible vasculitis.     

A case of idiopathic pulmonary fibrosis with chronic eosinophilic pneumonia

A 58 year-old man was admitted to our hospital with complaints of dyspnea and cough. Chest X-ray examination revealed diffuse nodular shadow, infiltration in the left upper and lower lobes, and volume loss. BALF showed increased number of cells, particularly eosinophils (65.6%). Eosinophilia (17-13%) was also seen. The histological examination of the TBLB specimen revealed irregular thickening and edema of alveolar septa. From these findings together with clinical features the case was diagnosed as eosinophilic pneumonia. Oral administration of prednisolone started with 30 mg. Within a week, eosinophilia in peripheral blood decreased to 1%, PaO2 increased to 87.5 Torr in room air, and CRP became negative. His chest X-ray film showed the disappearance of infiltrative shadows in the left upper and lower lobes. On the 19th day was prednisolone decreased to 20 mg. Laboratory data and chest roentgenogram showed exacerbation, and PaO2 gradually decreased to 62.9 Torr. Patchy shadows were seen in the right upper lobe. The dose of prednisolone was again increased to 60 mg/day, and was quite effective. The drug dosage was tapered by 10 mg/10 days to 40 mg/day, however all data exacerbated again. The clinical course seemed peculiar for eosinophilic pneumonia, however small reticulonodular shadows on chest X-ray did not change. Therefore, open lung biopsy was performed. The histology of the specimen showed fibrosis, lymphocyte infiltration and thickening of alveolar septa. The clinical course and histopathological findings led to a diagnosis of idiopathic pulmonary fibrosis accompanied with chronic eosinophilic pneumonia.     

A case of migratory air space infiltration after radiation therapy for breast cancer]

A 54-year-old woman underwent conserving surgery for right breast cancer, and received a cumulative dose of 50 Gy of radiation therapy to the remaining part of the right breast. About five months after the termination of irradiation, cough and low-grade fever developed. The chest radiograph showed an infiltrative shadow in the right lung field. Organizing pneumonia was identified in the transbronchial lung biopsy specimen. After prednisolone was given to the patient the clinical symptoms and infiltrates seen in the radiograph disappeared. In the course of tapering the prednisolone dose, new infiltrative shadows developed in the upper right lung and the left lung. The histologic changes were shown by transbronchial lung biopsy to be organizing pneumonia. The increased dose of prednisolone resulted in the rapid improvement of the clinical symptoms and chest radiograph abnormalities. This case suggests that breast radiation after conserving surgery for breast cancer may cause a pathologic process similar to that of bronchiolitis obliterans organizing pneumonia.     

Pulmonary complications resembling Wegener's granulomatosis in ulcerative colitis with elevated proteinase-3 anti-neutrophil cytoplasmic antibody

After a 17-year-old woman with ulcerative colitis was treated with granulocyte apheresis, chest imaging showed multiple nodular infiltrates, including one which seemed to have a cavity. Wegener's granulomatosis was suspected because proteinase-3 anti-neutrophil cytoplasmic antibody levels were increased. Transbronchial lung biopsy specimen showed nonspecific findings. Chest imaging showed clearing of pulmonary infiltrates without any therapy or discontinuation of mesalazine, which is known to cause lung toxicity. To the best of our knowledge, this is the first report of extra-intestinal pulmonary complications of ulcerative colitis with elevated proteinase-3 anti-neutrophil cytoplasmic antibody resembling Wegener's granulomatosis and spontaneous improvement.     

A case of HTLV-1 associated myelopathy with pulmonary involvement

A 70-year-old woman was admitted complaining of gait disturbance and difficulty in urination. Neurological examination showed myelopathy and both serum and CSF anti ATLA antibodies were positive. A diagnosis of HTLV-associated myelopathy (HAM) was made and steroid therapy was initiated. Chest X-ray film on admission showed no abnormality, but three months later, diffuse fine nodular and reticular shadows appeared in both lung fields. The patients had no respiratory symptom. The results of pulmonary function tests were normal, aside from a mild obstructive defect as indicated by reduced V25. Arterial blood gas was also normal. Bronchoalveolar lavage studies showed increased total cell counts and an increased proportion of T-cells. The histological findings of the transbronchial lung biopsy specimen were bronchiolitis and alveolitis. Subsequently, within the next eight months the abnormal shadows on chest X-ray cleared gradually on maintenance dosage of prednisolone, 10 mg/day. Possible relationships between HAM and the pulmonary lesions were discussed.     

A case of bronchioloalveolar cell carcinoma with bilateral diffuse interstitial infiltrative shadow during the treatment of Takatsuki's disease]

A 54-year old man treated with Takatsuki's disease was referred to us complaining of cough and excessive sputum. A chest roentgenogram showed bilateral diffuse interstitial infiltrative shadow. Chest CT showed banding shadows around the bronchioles and lobule-septum thickening in the right middle and both lower lung fields, and many small nodules in both lower lung fields. The histological diagnosis was adenocarcinoma replaced with one layer of bronchiolar epithelium, and partly bronchiolo-alveolar carcinoma. The patient received 3 courses of combination chemotherapy with docetaxel and cisplatin. After chemotherapy, the chest CT showed no change. The clinicopathological characteristics of this rare case included adenocarcinoma mixed with bronchioloalveolar carcinoma, in which radiography showed bilateral diffuse interstitial infiltrative shadow.