Multiple nodular pulmonary amyloidosis with multiple bullae]

We report a case of multiple nodular amyloidosis accompanied by multiple emphysematous bullae. The patient was a 72-year-old nonsmoking woman who presented with chronic cough and had a history of pneumothorax. Chest computed tomography disclosed multiple nodules up to 1 cm in diameter, and multiple small emphysematous bullae predominantly distributed in the middle and lower lung fields. Left lung biopsy was performed under thoracoscopy. The nodules were histopathologically diagnosed as amyloidoma. The mechanism of the accompanying bullae formation was also considered.     

A case of solitary nodular pulmonary amyloidosis]

A 64-year-old man without respiratory symptoms was introduced to our hospital because of a nodule of 20 mm in diameter found in the left lung in a periodic health examination. The chest radiograph and CT scan showed a well-defined nodule in the middle of the left lung field, and enlarged mediastinal lymph nodes. Lung cancer was suspected, and transbronchial and CT guided biopsies were performed, but did not lead to a definitive diagnosis. Since the patient refused further examinations, we carefully followed up the nodule in the chest radiograph. After a year and a half, the nodule and the lymph nodes became enlarged, and the patient was admitted to the hospital for a surgical biopsy. Video-assisted thoracoscopic surgery followed by thoracotomy was performed for both a biopsy and nodule resection. Histologically, the nodule tissue was rich in amorphous substances positively stained with Congo Red, which was consistent with amyloidosis. No findings of systemic amyloidosis or secondary amyloidosis were demonstrated.     

Sj?gren's syndrome with multiple bullae and pulmonary nodular amyloidosis]

We report a case of Sj?gren's syndrome with pulmonary involvement diagnosed by open lung biopsy. The patient was a 62-year-old woman with antiphospholipid antibody syndrome. Her chest radiograph and CT scan showed multiple bullae diffusely scattered throughout the lung. The open lung biopsy specimens revealed marked inflammatory mononuclear cell infiltration and nodular amyloid deposits in the bronchiolar walls. The mechanism of bulla formation appeared to be the check valve mechanism caused by the narrowing of the airway by the bronchiolitis. The patient was treated with oral corticosteroids, and her symptoms and laboratory findings became stable.     

Sj?gren's syndrome with multiple bullae due to pulmonary nodular amyloidosis]

We reported a case of Sj?gren's syndrome with pulmonary involvement diagnosed by video-assisted thoracoscopic lung biopsy. The patient was a 54-year-old woman with antiphospholipid syndrome. Her chest radiograph and CT scan showed multiple nodules with or within cystic lesions. The thoracoscopic lung biopsy specimens revealed nodular amyloid deposits associated with bronchiolitis. The mechanism of bulla formation appeared to be a check-valve mechanism caused by the narrowing of the airway due to bronchiolitis with mononuclear cell infiltration. She has no symptoms without respiratory failure or functional impairment, therefore we are following her closely without therapy.     

A case of primary antiphospholipid syndrome complicated with pulmonary hypertension]

We describe the case of 55-year old male with antiphospholipid syndrome (APS) who developed pulmonary hypertension without any thromboembolic episode. Multiple pulmonary perfusion defects suggestive of in situ thrombosis were observed. Hematological findings revealed microangiopathic hemolytic anemia and thrombocytopenia. These findings were improved by anticoagulant therapy. We monitored mean pressure of pulmonary artery (mPAP) and total pulmonary vascular resistance (TPR) before and after using vasodilator agents by Swan-Ganz catheter. mPAP and TPR showed improvement on treatment with oxygen supplementation therapy and Isosorbide administration. Previously 11 cases with APS complicated with pulmonary hypertension were reported. Majority of these patients have had recurrent venous thrombosis, particularly deep vein thrombosis often accompanied by pulmonary thromboembolism (8/11 cases, 72%). However in this case pulmonary hypertension with APS may be induced by in situ thrombosis in pulmonary micro vessels.     

Sj?gren's syndrome with solitary nodular pulmonary amyloidosis]

We describe a case of limited pulmonary amyloidosis with Sj?gren syndrome. A 58-yr-old woman was referred to our hospital because of an abnormal chest radiograph (solitary small nodule) that was examined to investigate the cause of a persistent cough. A chest CT revealed a solitary small nodule in the left lower lung field. The specimens obtained by thoracoscopic surgery showed AL (kappa) amyloid deposits with lymphoplasmacytic infiltrate. Immunofixation of the serum and concentrated urine failed to demonstrate monoclonal immunoglobulins, and no amyloid deposits in the stomach were detected. She was subsequently diagnosed as having primary Sj?gren's syndrome. Nodular pulmonary amyloidosis with Sj?gren syndrome is very rare condition, and most cases present multiple nodules. As far as is known, this is the first report of a solitary nodule in pulmonary amyloidosis with Sj?gren syndrome.     

Immunohistochemical and electromicroscopic study of a case of primary nodular pulmonary amyloidosis]

A 41-year-old man was hospitalized for further evaluation of an abnormal chest films which revealed a nodular shadow in the right middle lung field, which remarkably enlarged during one year. As no diagnostic procedures ruled out lung carcinoma of right S6, a right lower lobectomy was performed. Pathologically the nodules were composed of amorphous and eosionphilic materials which were diagnosed as amyloid by Congo-red stain and electron microscopic examination. Since there were no deposits in other organs and there was no abnormality of serologic and urinary protein analysis, a diagnosis of primary nodular pulmonary amyloidosis was established.     

Sj?gren's syndrome with multiple bullae and pulmonary nodular amyloidosis

We treated a patient with Sj?gren's syndrome associated with multiple bullae and pulmonary nodular amyloidosis, both of which were identified by open lung biopsy. The mechanism of bullae formation appeared to be narrowing of the airway, as a result of extensive inflammatory cell infiltration to the bronchiolar wall, which acted as the check valve mechanism. We believe this to be the first reported case of Sj?gren's syndrome accompanied by these two pulmonary manifestations.     

A case of aortitis syndrome complicated with amyloidosis, type AA]

We recently saw a patient who had aortitis syndrome associated with secondary amyloidosis. To our knowledge, she is the fourth report of this complication occurring in aortitis syndrome. In November 1985, the patient, a 18 year-old woman, was admitted to our hospital because of a high fever, back pain, abdominal pain and general fatigue. On physical examination, bruit was audible on the abdomen, bilateral radial artery was weakly palpable. Angiography showed the stenosis of bilateral carotid artery, subclavian artery, renal artery and superior mesenteric artery. From the above findings, she was diagnosed aortitis syndrome, and treatment was begun with prednisolone. However, she developed recurrently a high fever, chest pain, abdominal pain and exertional dyspnea. Laboratory findings at the active stage revealed the marked elevation of leukocytes, erythrocyte sedimentation rate and C-reactive protein. On her clinical course, the number of circulating thrombocytes was paralleled with the activity of the disease. On June 1988, she developed suddenly a high fever and severe pain of abdomen. Pathological findings of her stomach showed the deposition of amyloid protein A. Laboratory findings depicted the marked increment of thrombocytes, beta-thromboglobulin and platelet factor 4. These results suggest that circulating thrombocytes may play a role in product ion of amyloid protein.     

An unusual case of localized form of primary macroglobulinemia developing from a nodular primary pulmonary amyloidosis]

The authors reported here a case of primary pulmonary amyloidosis, which developed into a localized form of primary macroglobulinemia (PMG) 10 years later. A nodular shadow was pointed out on routine chest x-ray films of a 61-year-old Japanese male in 1977. In 1981, a diagnosis of nodular primary pulmonary amyloidosis was made by percutaneous lung biopsy. At that time, he suffered from signs and symptoms of chronic cold agglutinin disease (CCAD). Cold agglutinins were IgM-kappa antibodies. In 1986, serum immunoelectrophoresis demonstrated the presence of a small amount of monoclonal IgM-kappa. In 1987, the patient was readmitted because of pleural effusions. In the pleural effusion, the IgM level was 3,341 mg/dl and the titer of cold agglutinin was 32,000. Cytological examinations of pleural effusion showed the proliferation of lymphocytes, lymphoid cells and plasma cells. These cells showed the monoclonality of IgM-kappa by the peroxidase-antiperoxidase method. At post-mortem examination in March 1988, PA and PMG were found, but both lesions were localized only in the thoracic cavity. It is suggested that primary amyloidosis (PA) as well as CCAD and monoclonal gammopathy of undetermined significance may be one of the pre-neoplastic conditions of PMG.     

Spontaneous resolution of multiple nodular pulmonary AA amyloidosis

A 62-year-old man presented with a two-week history of dry cough. A chest computed tomography (CT) showed three nodular masses of soft tissue density without calcification or cavitary formation in the right lung. F-18 fluorodeoxyglucose PET/CT scan revealed high FDG uptake in two out of three pulmonary nodules. Transbronchial lung biopsy specimens consisted of amorphous eosinophilic deposits that were demonstrated to be amyloid because they were positive for Congo Red staining. After oxidation with permanganate solution, the Congo Red staining disappeared, indicating that this amyloid was amyloid A protein-derived type. There was no evidence of any systemic diseases. We diagnosed the patient as having multiple nodular pulmonary AA amyloidosis. The patient was conservatively managed without treatment, and the pulmonary nodules disappeared spontaneously three months later.     

干燥综合征继发支气管肺淀粉样变一例

干燥综合征是一种多系统自身免疫病,累及肺可表现为多种疾病,主要表现为气道异常、间质性肺炎和淋巴增生性疾病,还可表现为一些少见的疾病,如肺淀粉样变、肺淋巴瘤、肺栓塞和肺动脉高压等。本文报道1例干燥综合征继发支气管肺淀粉样变。     

FDG-PET findings of nodular pulmonary amyloidosis with a long-term observation]

A 60-year-old woman was referred to our hospital because of an abnormal chest radiograph in May, 2000. She was found to have rheumatoid arthritis in March, 1998, and pharmacologic therapy with anti-rheumatic drug was started. The chest CT scan revealed bilateral multiple lung nodular lesions of various sizes up to 30 mm. F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated a lesion with intense FDG activity in the right lung with a standardized uptake value (SUV) of 10.1. Fiberoptic bronchoscopy revealed no endobronchial lesions. Video-assisted thoracoscopic surgery was done to ascertain the pathological diagnosis. Histological examination showed that the pulmonary nodules were composed of amyloid A (AA) protein. Secondary Sj?gren syndrome was subsequently diagnosed. A diagnosis of localized nodular pulmonary amyloidosis with AA type amyloid protein was made, and therapy with anti-rheumatic drugs was continued. After six years of therapy, the size of pulmonary amyloidoma was reduced, and the accumulation of FDG returned to normal. We reported this interesting case in which FDG-PET apparently reflected the disease activity of pulmonary amyloidosis.     

A case of pulmonary alveolar proteinosis complicated with pneumoconiosis and myelodysplastic syndrome]

A 72-year-old man who had worked as a coal worker for 28 years and as a tunnel construction worker for 18 years was admitted because of fever, dyspnea, and appetite loss. Pneumoconiosis had been diagnosed when he was 64 years old and myelodysplastic syndrome at 71 years of age. After admission, the patient was treated with antibiotics and anti-fungal drugs but did not respond. Respiratory failure gradually worsened and he died. Autopsy specimens revealed that the cause of death was exacerbation of respiratory failure due to pulmonary proteinosis rather than pulmonary infection. This is a case of pulmonary proteinosis complicated with pneumoconiosis and myelodysplastic syndrome which, to our knowledge, is rare. We also considered the possibility that defective pulmonary macrophage function due to myelodysplastic syndrome and long-term silica inhalation played a part in the development of pulmonary alveolar proteinosis in this case.     

A case of primary Sj?gren's syndrome complicated with lung adenocarcinoma]

A 69-year-old woman presented with headache. Her chest radiograph and computed tomographic scans showed a mass shadow causing superior vena cava syndrome. Bronchofiberscopic examination was nonproductive. The serum value of carcinoembryonic antigen was highly elevated, so we made a presumed diagnosis of primary non-small lung cancer. She also complained of dry eyes and mouth. The elevated values of serum antibodies against SS-A and SS-B and further examinations resulted in a definitive diagnosis of primary Sj?gren's syndrome. Chemotherapy was not effective and she died 14 months later. Autopsy revealed that the mass shadow was a primary lung adenocarcinoma. At the age of 66 she suffered a refractory pneumothorax and her pulmonary cysts or bullae were surgically resected. Those lesions had bullae, emphysema, and alveolar septae thickened by infiltration of lymphoplasmacytic cells. Because she had complained of xerostomia for the last few decades, we associated the cysts with Sj?gren's syndrome. Thoracic CT scans at that time showed a nodule next to a cystic lesion. We raise a possibility that lung cancer might derive from cystic lesions associated with Sj?gren's syndrome.