A case of bronchocentric granulomatosis associated with Aspergillus that was difficult to differentiate from lung cancer]

We encountered a case of suspected bronchocentric granulomatosis (BCG) occurring in a COPD patient. Chest CT on admission showed a nodular shadow with indentation and a notch-like opacity in right S(3)a. Based on these findings, lung cancer was suspected. As we could not establish a definitive diagnosis, video-assisted thoracic surgery was performed. The histological findings of the resected specimen demonstrated BCG with clustering of fungal hyphae in the bronchioles. As the lesion was resected completely, we did not give additional treatment with an antifungal agent. It is difficult to differentiate BCG from lung cancer preoperatively. However, considering the homogeniecity of the nodule, it may be able to differentiate this type of lesion from lung cancer based on the feature. We report a rare case of a solitary nodule caused by BCG with Aspergillosis.     

A case of Carrington's chronic eosinophilic pneumonia associated with bronchocentric granulomatosis in an asthmatic patient

A case of Carrington's chronic eosinophilic pneumonia associated with bronchocentric granulomatosis in a young asthmatic woman is reported. Among the numerous forms of "eosinophilic lung", the characteristic features of these two diseases in asthmatic patients are briefly described. Concerning the association, the authors have found that some authors suggest a common nosological framework with many transitional forms.     

A case of bronchocentric granulomatosis presenting as a tumorous shadow on chest X-ray film]

A 57-year-old woman was admitted to our hospital with cough, sputum and abnormal chest X-ray. In summer, 1989, she developed cough which gradually worsened in autumn. In November, the chest X-ray revealed a tumorous shadow in the left suprahilar region. On admission, there were no symptoms of bronchial asthma. Chest X-ray revealed a subpleural tumorous shadow in the left upper lung field. X-ray findings suggested that the tumorous shadow in the suprahilar region moved to the left peripheral upper lung field. Left B1+2 orifice obstruction with necrotic tissue was seen on fiberoptic bronchoscopy. Transbronchial biopsies failed to yield specific diagnostic findings, except for bronchitis with exudate containing eosinophils. In February, 1990, she developed hemosputum and left chest pain. Chest X-ray showed consolidation in the left apical lung field. Left upper lobectomy was performed. Histological examination disclosed many granulomas with central necrosis around the bronchi, and aspergillus hyphae were seen. These findings are compatible with bronchocentric granulomatosis without asthma.     

Corticosteroid monotherapy in a case of bronchocentric granulomatosis with a two-year follow-up

Bronchocentric granulomatosis (BCG) is a rare disease. Because of the possibility of fungal infection, BCG has usually been treated with corticosteroids and antifungal agent. However, fungi are not detected in all BCG tissues. We report a case of proven BCG by open lung biopsy without fungi in a woman with corticosteroids monotherapy and two-year follow-up.KEYWORDS : Bronchocentric granulomatosis (BCG), fungal infection, open lung biopsy, corticosteroids, allergic diseaseBronchocentric granulomatosis (BCG), which was first described by Liebow (1) in 1973, is a rare disease characterized by inflammation that begins within and is centered around the walls of bronchioles and manifests as fever, cough, wheezing, dyspnea, and blood eosinophilia. It is considered to occur primarily in asthmatic patients and to be closely related to fumigatus infection (2-4).Because of the possibility of fungal infection, BCG is usually treated with corticosteroids and an antifungal agent (5). However, fungi are not detected in all BCG tissues. The question thus arises of whether antifungal agents should be used in addition to corticosteroids in these patients. We here report a case of biopsy-proven BCG without accompanying fungal infection in a woman who was treated with corticosteroids alone and followed-up for two years.     

Pulmonary tuberculosis and bronchocentric granulomatosis

Bronchocentric granulomatosis (BG) is an uncommon inflammatory lesion of unknown etiology defined on morphologic grounds by the presence of necrotizing granulomata centered on bronchi and bronchioles. We report the typical pathologic features of BG in a patient with tuberculosis. Mycobacterial and other types of infection should be excluded by appropriate stains and cultures in all patients with BG on lung biopsy, especially those who are nonasthmatic.     

A case of Wegener's granulomatosis with pachymeningitis]

A 62-year-old woman who had been receiving corticosteroid therapy for pachymeningitis since 1997 was admitted to our hospital when an abnormal shadow was noticed in her chest radiograph. In bronchial and nasal mucosal biopsies, the findings of a necrotic granulomatous lesion and vasculitis were compatible with Wegener's granulomatosis, although this is rarely seen with pachymeningitis. After further corticosteroid therapy together with cyclophosphamide treatment, the size of the thoracic X-ray shadow decreased. Methicillin-resistant Staphylococcus aureus (MRSA) was cultured from the sputum and the nasal fluid, and may have contributed to the advance of the disease in the airway. This case will require continuing careful observation.     

A case of limited Wegener granulomatosis with hypereosinophilia]

A 51-year-old female was admitted to Nagano Matsushiro General Hospital because of fever, cough and dyspnea on exertion. Her laboratory data revealed leukocytosis with hypereosinophilia, a high erythrocyte sedimentation rate and c-reactive protein. Chest radiography revealed an infiltration shadow with a cavity in the right upper lobe. A lung abscess was diagnosed and antibiotics were administered. Laboratory results showed improvement, but chest radiography continued to show cavities. She was admitted to our hospital because of fever, left pleural effusion and progression of cavities on chest radiographs. She showed no abnormalities of the upper airway or kidney, and was negative for c-antineutrophil cytoplasmic antibody (c-ANCA). Because a positive c-ANCA was seen on day 8 of hospitalization, L-type limited Wegener granulomatosis (WG) was diagnosed according to Gross et al. Prednisolone (PSL) was administered, which improved the anemia, eosinophilia and the cavities. On day 7 of PSL administration, of the left pneumothorax occurred as a complication caused by perforation of the left chest cavity, but her clinical course was good after a cavernectomy was performed. Some studies have reported that limited WG shows a negative c-ANCA, and that antibiotic therapy improves inflammation. The L-type of limited WG revealed a low-grade positive ratio and titer of c-ANCA. Moreover, L-type limited WG responds well to therapy. We therefore selected PSL administration only against L-type limited WG. We have reported L-type limited WG with eosinophilia and the negative effects of c-ANCA at an early clinical stage.     

A case of Wegener's granulomatosis associated with the syndrome of inappropriate secretion of ADH]

A 68 year-old woman was admitted with fever, productive cough and sore throat. A chest radiograph and a chest computed tomography showed multiple nodules in both lungs. Thoracoscopic lung biopsy was performed. The specimens showed vasculitis and geographic basophilic necrosis with palisading histiocytes, giant cells, and neutrophils. Wegener's granulomatosis was diagnosed. On the 5th hospital day, the serum sodium level was 128 mEq/l. Since secretion of antidiuretic hormone had continued despite a low plasma osmolarity, we diagnosed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and initiated oral prednisolone and cyclophosphamide. As a result, the symptoms and image findings were improved, and serum sodium level became normal. This case was considered to be SIADH secondary to Wegener's granulomatosis.     

A case of lymphomatoid granulomatosis with multiple thin-walled cavities]

We report a case of rapidly progressive lymphomatoid granulomatosis. A 48-year-old man was admitted because of hemoptysis and high fever. A chest radiograph and chest computed tomograms revealed multiple nodules, thin-walled cavities in the lungs, some containing ball-like masses. After admission, clinical studies, including percutaneous and transbronchial biopsies failed to provide sufficient evidence for proper diagnosis. Fluoro-deoxyglucose positron emission tomography (FDG-PET) showed strong accumulations of abnormal shadows in the lesions. A VATS biopsy was performed to make possible an exact diagnosis. The histological findings showed angiocentric lesions with infiltration of polymorphous cells, and were compatible with lymphomatoid granulomatosis (LYG). The respiratory failure progressed as the reticular shadows in the chest radiograph increased, so treatment with methylprednisolone pulse therapy (1,000 mg/day for 3 days) was started, and followed with prednisolone therapy (60 mg/day for 3 weeks). Initially, the symptoms improved, but gradually deteriorated, and the pulmonary nodules became enlarged. The patient finally died of progressive respiratory failure in addition to uncontrollable empyema with an MRSA infection. We attributed the cavity formation and ball-like masses in the cavities to the occlusion of small pulmonary arteries and tissue necrosis along the drainage bronchus.     

Asbestosis,endobronchialAspergillus infection,and bronchocentric granulomatosis presenting with hemoptysis

A patient with pulmonary asbestosis developed hemoptysis and a lesion on chest radiograph secondary to bronchocentric granulomatosis (BG) associated withAspergillus fumigatus. Although pulmonaryAspergillus infection is a known complication of asbestos exposure, BG has not been previously reported in this disorder. This patient’s case further documents thatAspergillus hyphae may be present in BG unassociated with allergic bronchopulmonary aspergillosis. A wide range of processes affecting airways, including asbestos exposure, may be associated with bronchocentric granulomatosis.     

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.     

Acute interstitial nephritis with uveitis. A case report]

Dobrin et al described in 1975 the first case report with acute renal failure due to tubulo-interstitial nephritis accompanied by uveitis and an inflammatory syndrome. The young adults are frequently involved and the complete resolution is usually obtained after corticosteroid treatment; this contrasts with the tendency towards relapse of the uveitis. The etiology and the pathogenesis of this syndrome remain unknown. We report the case of a woman which the renal function was incompletely reversible with corticosteroids.     

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 with a giant solitary pulmonary mass]

A 44-year-old woman was admitted to hospital with a history of high fever, cough, and weight loss. Her chest X-ray film showed a giant solitary pulmonary mass in the left lower lobe. After admission, she developed bilateral eyeache and left exophthalmos. CT film of the head showed a massive lesion in the sinuses. Biopsy specimens obtained from both massive lesions showed either epithelioid granulomas with Langhans' giant cells or necrotising vasculitis. Antineutrophil cytoplasmic antibodies, which have high sensitivity and specificity for active WG, were found. The diagnosis of WG was thus made.     

A case of refractory Wegener's granulomatosis successfully treated with high-dose methotrexate]

We report a case of generalized Wegener's granulomatosis (WG) successfully treated with high-dose methotrexate (MTX) and predonisolone (PSL). A 35 year-old men had complaints of auditory disturbance and nasal hemorrhage. Diagnosis of WG was made based on positive proteinase-3 anti-neutrophil cytoplasmic antibody (PR3-ANCA), lung nodules, and focal necrotizing glomerulonephritis revealed by renal biopsy. Treatment with cyclophosphamide (CY) and PSL for 3 months was ineffective for the lung nodules. Then, CY was changed to high dose MTX (18mg/week), and his lung lesions improved. In Japan, it is unusual to treat WG with high-dose MTX, but this treatment may be useful in CY-resistant WG.     

A case of limited form of Wegener's granulomatosis associated with persistent high titer of serum antineutrophil cytoplasmic antibodies]

A thirty-six year-old male was admitted to our hospital because of a two year history of orbital mass. He had experienced inflammatory tumor of right lung six years before admission. Both the lung tumor and orbital mass were revealed as chronic panarteritis with diffuse necrotizing lesions. He was treated with low dose prednisolone, but the orbital mass gradually enlarged. We detected fluorescent cytoplasmic pattern of antineutrophil cytoplasma antibodies (ANCA) by the indirect immunofluorescence method (IF). We ascertained this case to be a limited form of Wegener's granulomatosis (WG) by histopathological finding and positive ANCA. The value of ANCA, estimated by enzyme linked immunosorbent assay (ELISA) was rather higher than that of generalized form of WG in other reports. There was no reduction of the size of orbital mass and the value of ANCA despite strong immunosuppressive therapy. In this case, the value of ANCA didn't represented disease activity. The presence of histopathological findings such as those observed in the present patient, i.e., abnormally high ANCA levels not associated with disease severity or progression, which are not in accordance with those conventionally reported, was considered very interesting in terms of the prevailing beliefs concerning involvement of ANCA in the mechanism of onset of WG.     

A suspected case of negative PR 3-ANCA Wegener's granulomatosis associated with marked endobronchial lesion and systemic angitis]

A 72-year-old woman had been diagnosed as having organizing pneumonia at another hospital. She was treated with antibiotics, bronchodilators and prednisolone, but her cough, fever and inflammatory signs were not resolved completely. She was admitted to our hospital for further evaluation of the lung lesion. The chest CT scan revealed narrowing of the segmental bronchus and the serum samples showed elevated MPO-ANCA but negative PR 3-ANCA levels. Physical examination also revealed marked bilateral episcleritis and eyeground angitis. Fiberoptic bronchoscopy showed bilateral marked stenotic segmental bronchi associated with mucosal erosions and small nodular lesions. Bronchial and renal biopsy specimens demonstrated marked angitis in the bronchial arteries and glomeruli. A generalized form of Wegener's granulomatosis (WG) was diagnosed and was treated with 60 mg/day of prednisolone, 50 mg/day of cyclophosphamide and 4 g/day of sulfamethoxazole-trimethoprim, which resulted in a marked improvement of the clinical symptoms and endobronchial lesions. In WG, the incidence of endobronchial involvement is 16 to 55% and that of positive serum MPO-ANCA alone is only 14.3%. The present case of WG was considered a rare case with WG which because of the MPO-ANCA-positive but PR 3-ANCA-negative findings associated with marked endobronchial lesions and systemic angitis.