1/74. lung adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome.We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary sjogren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary sjogren's syndrome by a labial gland biopsy and classical clinical features including xerophthalmia, xerostomia and immunoserological findings. Pathological findings including immunohistochemical studies in a surgically resected lung revealed adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome. There was no evidence of malignant lymphoma in the lymph nodes or resected lung tissue. Pulmonary involvement of sjogren's syndrome is now regarded both clinically and histopathologically as a wide spectrum of lymphoproliferative disorders ranging from benign to malignant. However, lung cancer associated with primary Sjogren's syndrome, as in our case, has apparently not been reported previously.- - - - - - - - - - ranking = 1keywords = interstitial (Clic here for more details about this article) |
2/74. Mesangial nephropathy in sjogren's syndrome.We describe a 36-year-old woman with Primary sjogren's syndrome (PSS). purpura, corneal perforation, metabolic acidosis, decreased glomerular filtration, hypokalemia, hyposthenuria, and polyuria were present. Chronic renal insufficiency and renal tubular acidosis type I were diagnosed. kidney biopsy revealed mesangial glomerulonephritis, interstitial nephritis, and tubular atrophy. Replacement treatment with saliva, tears, and potassium citrate was started. She was given prednisone and cyclophosphamide. This would be the first description of PSS, mesangial glomerulonephritis, and chronic renal insufficiency.- - - - - - - - - - ranking = 0.16666666666667keywords = interstitial (Clic here for more details about this article) |
3/74. Clinically significant and biopsy-documented renal involvement in primary Sjogren syndrome.Clinically significant renal involvement in patients with primary Sjogren syndrome (pSS) has been described previously only in isolated case reports. The prevalence and significance of the 2 described syndromes, interstitial nephritis (IN) and glomerulonephritis (GMN), are not well known. In a cohort of 471 patients with pSS who were followed for a mean of 10 years, 20 patients (4.2%) developed overt renal disease. Eighteen patients underwent a percutaneous renal biopsy; 2 patients declined. Ten patients had IN, 8 patients had GMN, and 2 patients presented with both entities. Glomerular histology disclosed changes compatible with membranoproliferative GMN in 5 patients and mesangial proliferative GMN in 4 patients. patients with IN had a younger disease onset compared with patients with GMN (mean, 36.8 compared with 46.0 yr, p 5 0.063). patients with GMN had longer disease duration compared with patients with IN (mean, 2.2 compared with 8.0 yr, p 5 0.001). The majority of patients with GMN (80%) had mixed monoclonal cryoglobulinemia IgMk (type II) and lower complement c4 levels. Two patients (both with GMN) developed chronic renal failure requiring hemodialysis. overall, clinically significant renal involvement is infrequent in pSS. IN occurs early in the disease process, while GMN is a late sequela and may have a less favorable prognosis.- - - - - - - - - - ranking = 0.16666666666667keywords = interstitial (Clic here for more details about this article) |
4/74. sjogren's syndrome, cavitating lung disease and high sustained levels of antibodies to serine proteinase 3.A case is described involving sjogren's syndrome, high sustained levels of antibodies to serine proteinase 3, and cavitating lung disease. Possible diagnoses accounting for this unusual combination include a novel association of sjogren's syndrome and Wegener's granulomatosis (suggested by the high and sustained levels of antibodies to serine proteinase 3) or a rare presentation of bronchiolitis obliterans organising pneumonia. Identification of the true nature of the patients illness facilitated more active management and a swift resolution of the clinical problem.- - - - - - - - - - ranking = 0.012327631985901keywords = pneumonia (Clic here for more details about this article) |
5/74. Interstitial pneumonia complicated by sjogren's syndrome, Hashimoto's disease, rheumatoid arthritis and primary biliary cirrhosis.A 66-year-old woman diagnosed as having Hashimoto's disease and rheumatoid arthritis manifested interstitial pneumonia. We diagnosed sjogren's syndrome and primary biliary cirrhosis as complications in this case. Steroid therapy was relatively effective for the interstitial pneumonia which was in an active state; however, during tapering of the steroid, there was a relapse and also severe dry throat. cyclophosphamide was added and was effective in the prevention of recurrence. Even after discontinuation of steroid therapy, her general condition is stabilized. It is very important to carefully investigate other organ involvement as a prognostic factor in cases in which there are multiple autoimmune diseases.- - - - - - - - - - ranking = 2.3744391132788keywords = interstitial pneumonia, interstitial, pneumonia (Clic here for more details about this article) |
6/74. An autopsy case of primary biliary cirrhosis with severe interstitial pneumonia.A 74-year-old woman was admitted to our hospital because of interstitial pneumonia. She had a 14-year history of primary biliary cirrhosis (PBC) diagnosed histologically, with a positive test for anti-mitochondrial antibodies and elevated biliary enzyme activity. She also had a 7-year history of rheumatoid arthritis and a 26-year history of sjogren's syndrome. Though the symptoms of these complications improved, the interstitial pneumonia deteriorated very quickly and the patient died of respiratory failure due to acute exacerbation of interstitial pneumonia when the activity of PBC decreased. We report this case because it is relatively rare for PBC to be complicated by severe interstitial pneumonia, and it may offer insight into the etiology of these diseases.- - - - - - - - - - ranking = 9.3005143413408keywords = interstitial pneumonia, interstitial, pneumonia (Clic here for more details about this article) |
7/74. Two cases of sjogren's syndrome with multiple bullae.Here, we report two rare female cases of sjogren's syndrome with multiple bullae, involving a 66- and a 51-year-old. Neither had any obvious pulmonary complaint. Chest radiographs and high-resolution CT (HRCT) scans showed interstitial linear and nodular opacities and multiple bullae. In the first case spirometry indicated an obstructive change judged by FEV1.0 and V50/V25. In both cases, histologic examination of the lung revealed thickening of alveolar septa and interstitial mononuclear cell infiltration. In the first case the bullae decreased in size with corticosteroid treatment. Airway narrowing due to peribronchiolar mononuclear cell infiltration causes a check-valve mechanism, which may lead to bullae formation. Although a rare occurrence, it is important to recognize that cystic or bullous lung disease can accompany sjogren's syndrome.- - - - - - - - - - ranking = 0.33333333333333keywords = interstitial (Clic here for more details about this article) |
8/74. Primary sjogren's syndrome associated with inclusion body myositis.OBJECTIVE: To describe three new patients with inclusion body myositis (IBM) associated with primary sjogren's syndrome (pSS) and summarize the clinical and serological picture for all six patients reported so far. patients AND methods: Three out of 518 (0.6%) pSS patients followed in our department over a period of 15 yr (1985-2000) also presented IBM. The diagnosis was based on histological criteria. Previous reports described four more patients with IBM associated with pSS. Five out of six were females; the mean age of IBM onset was 53.3 /-14.0 yr and the disease duration 8.0 5.8 yr. RESULTS: Four out of six patients presented IBM several years after the onset of pSS. Four out of six patients presented extraglandular manifestations, such as arthralgias, vitamin B12 deficiency, interstitial kidney disease and biliary cirrhosis. The serological picture did not differ significantly from that observed in pSS alone. CONCLUSION: Although rare, IBM may be seen in patients with pSS and it affects predominantly women. The clinical and pathological pictures do not differ from those observed in idiopathic IBM. A common autoimmune pathway in these two diseases, but no definite conclusions can be drawn.- - - - - - - - - - ranking = 0.16666666666667keywords = interstitial (Clic here for more details about this article) |
9/74. Lymphocytic interstitial pneumonitis associated with Epstein-Barr virus in Systemic Lupus Erythematosus and sjogren's syndrome. Complete remission with corticosteriod and cyclophosphamide.Lymphocytic interstitial pneumonitis (LIP) is characterized by diffuse bilateral pulmonary infiltrations in both lower lobes. Pleomorphic lymphohistiocytes including mature lymphocytes, variable admixture of plasma cells and other mononuclear cells infiltrate within the pulmonary interstitium, ranging from widened septa to confluent masses. We report a case of LIP associated with Epstein-Barr virus in a patient with SLE and sjogren's syndrome. A 50-year-old woman was admitted with insidious onset of progressive dyspnea for 20 days. She suffered from arthritis 10 years earlier without specific diagnosis. A radiography of chest has showed bilateral consolidative infiltrations with pleural effusion in both lower lung fields. Open lung biopsy documented lymphocytic interstitial pneumonitis which expressed Epstein-Barr virus genome in situ hybridization study. Following corticosteroid and cyclophosphamide therapy, clinical symptoms and radiologic infiltrations gradually remitted.- - - - - - - - - - ranking = 1keywords = interstitial (Clic here for more details about this article) |
10/74. A case of sjogren's syndrome or multiple sclerosis? A diagnostic and therapeutic dilemma.BACKGROUND: Primary sjogren's syndrome (SS) with central nervous system involvement and antiphospholipid syndrome have been reported to mimic multiple sclerosis (MS). SS has also been found in patients diagnosed with definite MS. patients AND methods: A Chinese lady developed multiple relapsing neurological events involving the brain and spinal cord from the age of 40 years. magnetic resonance imaging of the brain and spinal cord during acute relapses showed increased T2 signals and spinal cord edema. She later developed positive anti-nuclear antibody and anti-Ro autoantibody followed by sicca symptoms, confirming the diagnosis of SS. She died from aspiration pneumonia following pseudobulbar palsy shortly after SS was diagnosed. CONCLUSIONS: patients with MS-like disease and autoantibodies should be carefully evaluated for other systemic autoimmune diseases as the treatment and prognosis differ from MS. In particular, sicca symptoms should be sought as they often go unnoticed by patients. This is especially important in Orientals in whom MS is relatively uncommon.- - - - - - - - - - ranking = 0.012327631985901keywords = pneumonia (Clic here for more details about this article) |
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