1/37. Diffuse alveolar damage in BCGosis: a rare complication of intravesical bacillus Calmette-Guerin therapy for transitional cell carcinoma. Intravesical administration of bacillus Calmette-Guerin is a relatively simple procedure used in the treatment of superficial transitional cell carcinoma of the urinary bladder. It is usually well tolerated with few major side effects. In rare instances, systemic complications can result in death. The usual autopsy finding is that of caseating and non-caseating granulomata in the affected organ. We report the second case of BCGosis in which granulomata together with acute and organising phase diffuse alveolar damage were found in the lungs. ( info) |
2/37. Necrotizing sarcoid granulomatosis in a 14-yr-old female. A case of a 14-yr-old female with necrotizing sarcoid granulomatosis (NSG) is presented. She was referred because of chest pain and malaise, and radiography revealed multiple pulmonary nodules. Her history showed seasonal sensitization to aeroallergens and hay fever. Infectious agents or malignancies did not characterize these nodules. However, she was treated with macrolide antibiotics because of suspected infection with chlamydia pneumoniae. Open lung biopsy showed histological findings of NSG, with epithelioid granulomatous inflammation, including giant cells, and vasculitis. No further treatment was performed, and symptoms disappeared within a few weeks. The chest radiograph showed gradual improvement. The aetiology of NSG is poorly understood, and is postulated to represent either sarcoidosis or rare forms of pulmonary vasculitis such as Wegener's granulomatosis or the churg-strauss syndrome. In the case presented, a coincidence of infection with chlamydia pneumoniae suggests an involvement of infectious agents in the pattern of formation of immune complexes in the aetiology of NSG. ( info) |
3/37. Sarcoid-like pulmonary disorder in human immunodeficiency virus-infected patients receiving antiretroviral therapy. We report two cases of hiv-infected patients who presented with diffuse interstitial micronodular lesions on chest X-ray after institution of protease inhibitor-containing highly active antiretroviral therapy (HAART). Granulomatous pulmonary disorder mimicking sarcoidosis was diagnosed on histopathological studies revealing noncaseating granuloma and bronchoalveolar lavage analysis showing an intense CD4( ) lymphocyte alveolitis. Causative agents such as infectious organisms and environmental compounds were excluded. The relationship between sarcoid-like reaction and immune reconstitution under HAART is discussed. ( info) |
4/37. Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy. A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV dna was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56 lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8 T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis. ( info) |
5/37. Bronchocentric granulomatosis due to aspergillus terreus in an immunocompetent and non-asthmatic woman. This is the first report of bronchocentric granulomatosis due to aspergillus terreus in a healthy and non-asthmatic 74-year-old Japanese woman. Following identification of the fungus, oral itraconazole therapy was begun after intrabronchial infusion of amphotericin b. No recurrence has occurred after treatment for 24 months. We should consider the possibility of bronchocentric granulomatosis including aspergillus terreus, when an intrabronchial lesion is found even in a healthy and non-asthmatic person. Oral itraconazole after intrabronchial infusion of amphotericin b seems to be effective in such cases. ( info) |
6/37. Bronchocentric granulomatosis as a first clinical manifestation in an adult patient with p67phox deficiency. We report on a case of adult chronic granulomatous disease which first manifested as a pulmonary mass, and was histologically diagnosed as bronchocentric granulomatosis associated with aspergillosis in a patient with a deficiency of p67phox and a low oxidative response. Antifungal treatment was required for clinical resolution. copyright copyright 1999 S. Karger AG, Basel ( info) |
7/37. Severe pulmonary disease in association with Crohn's disease in a 13-year-old girl. Pulmonary manifestations of Crohn's disease are infrequent in adults and even less common in children. Our literature search found only a few cases of Crohn's disease causing pulmonary manifestations in children. We report on the case of a 13-year-old girl in whom severe pulmonary disease was found four years after the onset of Crohn's disease. Open lung biopsy uncovered bronchiolitis obliterans and granulomatous lung disease. Aggressive treatment has yielded gradual improvement. This case emphasizes the importance of recognizing the association, the differential diagnosis, and treatment implications. ( info) |
8/37. Hard metal alveolitis accompanied by rheumatoid arthritis. Hard metal lung diseases (HML) are rare, and complex to diagnose. We describe the case of a patient with allergic alveolitis accompanied by rheumatoid arthritis. A sharpener of hard metal by trade, our patient was a 45-year-old, nonsmoking Caucasian female who experienced symptoms of cough and phlegm, and dyspnea on exertion. Preliminary lung findings were inspiratory rales in both basal areas, decreased diffusion capacity and a radiological picture resembling sarcoidosis. A high-resolution computed tomography scan indicated patchy alveolitis as well. An open lung biopsy revealed non-necrotizing granulomas consisting of epitheloid cells and surrounded by lymphocytes, plasma cells and a few eosinophils. These cells also occupied the thickened alveolar interstitium. macrophages in the alveolar spaces, some of them multinuclear, contained dust particles. Hard metal alveolitis is clinically well known and, in this patient, has been described histologically. After the patient had quit working with hard metal and following corticosteroid therapy, pulmonary symptoms and signs were relieved. During this recovery period, however, she contracted rheumatoid arthritis. ( info) |
9/37. Necrotizing pulmonary granulomata in a marijuana smoker. We describe the case of a heavy marijuana and tobacco smoker who presented with progressive exertional dyspnea of 2 months' duration, and bilateral nodular lung infiltrates. Examination of the lung fields was normal, and lung function tests showed mild airflow obstruction with moderately reduced gas transfer. BAL returned green-black fluid consisting predominantly of macrophages laden with carbon pigment. Thoracoscopic lung biopsy showed miliary necrotizing granulomata with an alveolar exudate of carbon-laden macrophages within macroscopically blackened lung. The differential diagnosis of pulmonary granulomata in this patient is discussed. ( info) |
| patients with common variable immunodeficiency (CVID) are occasionally recognized to have a concurrent noncaseating granulomatous disease. The granulomatous disease (GD) associated with CVID shares many clinical properties typical of sarcoidosis. Some investigators speculate that the GD-CVID is actually sarcoidosis that is expressed atypically because of the patient's immunodeficiency. Clinical differences, however, have led other investigators to speculate that the GD-CVID is a distinct "sarcoid-like" granulomatous process. ( info) |