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 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 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.     

Pulmonary alveolar proteinosis noted in a patient with thymoma

A 74-year-old woman had general fatigue and mild fever in August 2004. Her chest X-ray showed slight ground glass opacities in the upper and middle lung fields of both lungs. Though she was prescribed antibacterial drugs, the abnormal shadows on chest X-ray did not improve. The chest CT showed ground glass opacities and reticular shadows with thickened alveolar septa (crazy-paving appearance) in both lungs, and a clearly defined mass in the anterior mediastinum. She underwent thymo-thymectomy with wedge resection of the upper lobe of the left lung. Anterior mediastinum tumor was pathologically diagnosed as thymoma. Lung biopsy demonstrated alveoli filled with SP-A positive granular materials, and we diagnosed pulmonary alveolar proteinosis. About 1 month after operation, the shadows on chest CT showed improvement. We think there might be some relationship between thymoma and pulmonary alveolar proteinosis.     

Bronchioloalveolar carcinoma complicated by a lesion resembling bronchiolitis obliterans organizing pneumonia in the opposite lung]

A 50-year-old woman with abnormal shadows in her chest radiograph was admitted for a more detailed examination in April 2000. A chest CT scan showed infiltrates surrounded by ground-glass opacities in the right upper lobe, and ground-glass opacities with partial infiltrative changes in the left lower lobe. A diagnosis of adenocarcinoma like bronchioloalveolar carcinoma (BAC) was made for the lesion in the right upper lobe by means of a transbronchial biopsy. However, the specimen from the left lower lobe showed nonspecific changes, and bronchiolitis obliterans organizing pneumonia (BOOP) or a metastatic lesion from the BAC was suspected. We prescribed steroids for the short term. Since the lesion in the left lobe disappeared, we performed a right upper lobectomy. In cases of primary lung carcinoma, BOOP in the opposite lung is rare and it was difficult to decide on the operability. We concluded that steroid therapy is useful for distinguishing between inflammatory and tumorous lesions in cases in which a detailed examination is difficult.     

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.     

Pulmonary lymphoma of large B-cell type mimicking Wegener's granulomatosis

A 27-year-old man with a primary pulmonary lymphoma of large B-cell type is described. Symptoms involved both the upper and lower respiratory tract. A chest roentgenogram showed a dense mass with cavitation. Transbronchial biopsy specimens revealed no atypical cells, rather they demonstrated granulomatous infiltration and vasculitis consistent with but not conclusively diagnostic of Wegener's granulomatosis. The pulmonary mass became smaller after sulfamethoxazole-trimethoprim therapy. These features suggested Wegener's granulomatosis. However, an open biopsy specimen was diagnostic for diffuse lymphoma of large B-cell type. High-grade pulmonary lymphoma should be considered in the differential diagnosis of patients with clinical and pathologic features suggesting Wegener's granulomatosis.     

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.     

Wegener's granulomatosis in a woman with asymptomatic primary biliary cirrhosis

A 60-year-old woman was admitted to our hospital in June 1985, complaining of fever, cough and right lower chest pain, with a five-year history of asymptomatic primary biliary cirrhosis. Chest X-ray on admission showed an infiltrative shadow in the right lower lung field. She was first treated with various antibiotics unsuccessfully. Hemoptysis continued. Dyspnea and anemia appeared. Chest X-ray 17 days after admission showed multiple infiltrative shadows in the both lung fields. She was treated with steroid pulse therapy successfully. During prednisolone treatment decreasing nodular shadows with cavities appeared on chest X-ray. An open lung biopsy was performed in March 1986. The histologic findings showed a necrotizing vasculitis with granuloma and perivascular fibrosis. She was treated with prednisolone and prednisolone-azathioprine therapy unsuccessfully, but successfully with prednisolone-cyclophosphamide therapy. This case was a rare case of Wegener's granulomatosis with transition from fulminant type to granulomatous type. No similar case of Wegener's granulomatosis with asymptomatic primary biliary cirrhosis has been reported in the literature.     

A case of sarcoidosis manifesting as annular opacities after multiple nodular shadows]

A 40-year old man was admitted to our hospital because nodular shadows in the right upper lung field had shown deterioration on chest radiographs during a regular check-up (Sep. 28, 2000). Transbronchial lung biopsy specimens showed findings compatible with sarcoidosis. During follow-up, the nodular shadows became bigger, spread in both lung fields and turned into multiple annular opacities. In December, lung biopsy specimens obtained by VATS showed central areas of ground-glass attenuation corresponding to areas of alveolar septal inflammation, in contrast to the denser periphery, where polypoid organized granulation tissue in peripheral air spaces predominated. After 2 months, the opacities disappeared without treatment. In our case, the annular opacities on CT corresponded histologically to BOOP. It is interesting to note the relationship between sarcoidosis and BOOP.     

A case of primary pulmonary MALT lymphoma as a nodular shadow on CT scan, and relapsed with diffuse micronodular shadows after surgical resection at 7 years ago]

A 76-year-old man without symptoms was admitted to our hospital for investigation of an abnormal chest shadow in 1994. His chest radiograph showed a nodular shadow in the lingual lobe. Segmentectomy was performed and the histological diagnosis was MALT (mucosa-associated lymphoid tissue) lymphoma. In June 2001, there were no abnormal shadows on the chest radiograph or in 10 mm slice CT. However, high-resolution CT with 2-mm slice thickness revealed diffuse micronodular shadows in both lungs. A relapse of MALT lymphoma was diagnosed by immunohistochemical analysis of tissue specimens obtained by transbronchial lung biopsy. It was observed that the patterns of radiological findings in primary pulmonary MALT lymphoma cases may differ between the initial state and a relapse.     

High resolution CT of Wegener's granulomatosis--case reports

Computed Tomography (CT) of the chest was performed in three patients with Wegener's granulomatosis to define the anatomical basis of pulmonary involvement. Nodules, masses with cavitation, and areas of parenchymal opacities were typical manifestations on CT images. An extremely wide variety of radiologic findings were demonstrated in addition to the commonly described cavitary nodules.     

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.     

Wegener's granulomatosis complicated with intestinal ulceration

We report the case of a 32-year-old man who developed Wegener's granulomatosis complicated with refractory intestinal ulceration. In August 2001, he presented with a high fever, nasal bleeding, and bilateral leg numbness. These symptoms worsened, which prompted him to consult his home doctor on February 18, 2002. In spite of treatment with antibiotics, his symptoms did not improve. Furthermore, abdominal pain and melena occurred as additional symptoms in March 2002. He was admitted to our hospital on April 5, 2002. A deformed nose condition (the so-called saddle nose) was observed at this time. Laboratory data showed a high erythrocyte sedimentation rate (103 mm/h) and a high level of serum C-reactive protein (14.98 mg/dl), and hematuria and proteinuria were also observed. The patient was positive for an antineutrophil cytoplasmic antibody specific for proteinase-3 (PR3-ANCA). A chest computed tomography (CT) scan revealed multiple pulmonary nodules in the lung field. A biopsied-specimen from the nasal mucosa showed necrotizing granulomatosis with giant cells. Together with his symptoms and the laboratory and pathological findings, the patient was diagnosed as having Wegener's granulomatosis. A colon fiberscopy showed multiple ulcerations with bleeding from the terminal ileum to the ascending colon, and nodular lesions at the terminal ileum. We started a combination therapy of prednisolone (60 mg/day) and cylophosphamide (100 mg/day) orally. The patient's gastrointestinal symptoms disappeared and abnormal serological indicators improved. Although Wegener's granulomatosis complicated with refractory intestinal ulceration is rare, this case indicates that the gastrointestinal region is also a target organ of Wegener's granulomatosis.     

Aortitis syndrome associated with ulcerative colitis, preceded by pulmonary infarction involvement]

A 21-year-old woman with a 6-year history of ulcerative colitis admitted to our hospital with chest pain, cough and fever of unknown origin in August 1998. On admission, laboratory data showed positive inflammatory signs. A chest radiograph and chest computed tomogram (CT) revealed nodular shadows in the right upper lung field. Fifty days after admission, hypertension developed and a bruit was audible in the neck and the upper abdomen. Digital subtraction angiography showed stenosis in carotid, renal and right upper pulmonary arteries. On the basis of these results, a diagnosis of aortitis syndrome was made. Moreover, these findings indicated pulmonary infarction in the right upper lobe due to aortitis syndrome. Aortitis syndrome preceded by pulmonary infarction involvement is very rare. Autoimmune disorders may have been involved in this case because of the association with ulcerative colitis.     

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 Wegener's granulomatosis with necrotizing granuloma limited to intraorbital lesions]

A patient of Wegener's granulomatosis (WG) who developed intraorbital tumor without pulmonary and renal involvement was reported. A 52-year-old male has suffered from visual disturbance and right proptosis. Administration of betamethasone resulted in a temporary relief of his symptoms. However, following the reduction of steroid dosage, severe recurrence of ocular symptom occurred and his right sight was completely lost. On admission to our hospital, there were swollen eyelids and saddle nose. The CT scan demonstrated intraorbital tumors bilaterally associated with chronic sinusitis. The repeated biopsies of nasal mucosal lesions disclosed presence of a giant cell granuloma with necrotizing vasculitis. A diagnosis of WG had been made, so intermittent high-dose intravenous cyclophosphamide therapy were initiated. The size of the bilateral intraorbital tumors reduced with continuous cyclophosphamide therapy. At the last observation during 22 months after the initiation of cytotoxic therapy, there was no evidence of clinical and radiological recurrence. Three cases of WG with intracranial tumors in our hospital showed multiple cranial nerve palsy and poor response to corticosteroid therapy. The "limited form" without renal involvement has been known as a subset of WG with a relatively good prognosis. This report suggest the existence of further limited form with intracranial granuloma as a cardinal presentation of WG. Although the extent of granulomatous lesions are limited and progression is slow, the treatment by corticosteroid alone is not sufficient and as the systems form of WG, combined therapy with cyclophosphamide appeared to be indicated.     

Pulmonary lymphomatoid granulomatosis radiologically mimicking interstitial pneumonia]

We report a rare case of pulmonary lymphomatoid granulomatosis radiologically mimicking interstitial pneumonia. A 57-year-old man was admitted to our hospital because of chest bilateral reticular shadow with sustained cough and breathlessness for 10 years. Chest CT scans showed multiple ground-glass opacities, traction bronchiectasis and cystic change in both lungs, in addition to hilar and mediastinal lymphadenopathy. A histopathologically diagnosis of pulmonary lymphomatoid granulomatosis (angiocentric immunoproliferative lesion, grade 1) was made by thoracoscopic lung biopsy. In this case, serological and immunohistochemical analyses did not show Epstein-Barr virus infection. No clinical or radiological deterioration has been observed thereafter despite no medication.     

Case of bronchocentric granulomatosis that became recognized in the course of allergic bronchopulmonary aspergillosis]

A 56-year-old man with allergic bronchopulmonary aspergillosis (ABPA) was admitted due to the appearance of nodular opacities in the right upper lung field on chest radiography, after discontinuing itraconazole and clarithromycin on the suspicion of possible hepatic adverse effects. Chest CT scans on admission revealed nodular opacities in the right S3 and lingula bronchus, and bilateral bronchiectasis with mucoid impactions. A specimen obtained by transbronchial lung biopsy showed complete replacement of bronchioles by necrotizing granulomatous inflammation, containing the diagnosis of bronchocentric granulomatosis. Treatment with corticosteroids and micafungin sodium resulted in marked resolution of nodular opacities and mucoid impacts. This case suggests that abrupt cessation of antifungal agents and macrolides may provoke acute exacerbation of ABPA and development of bronchocentric granulomatosis.     

Pulmonary malignancy associated with granulomatous vasculitis in the maxillary sinus

Summary A 63-year-old man presented with pulmonary infiltrates and maxillary sinusal opacities. Although necrotizing vasculitis and granulomatous inflammation were present in nasal mucosa, pulmonary biopsy revealed adenocarcinoma. Simultaneous appearance of granulomatosis in the maxillary sinus and necrotic lung nodule does not necessarily mean that the lung abnormalities are also granuloma. This case report suggests that differential diagnosis between Wegener's granulomatosis and lung carcinoma may be difficult.