1/50. Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy.We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
2/50. Focal parotid necrosis in systemic lupus erythematosus: case report and review of the literature.Systemic lupus erythematosus (SLE) is a disease that may affect a number of organ systems, particularly the joints, skin, kidneys, heart, lungs, and immune system. Salivary gland involvement is usually associated with sjogren's syndrome, in which lymphocytic acinar infiltrates predominate histologically. We present the case of a 29-year-old woman with SLE who developed bilateral parotid enlargement with a unilateral focus of parotid necrosis that was consistent with a cystic mass on computerized tomography. A biopsy of this lesion was histologically similar to a cervical lymph node biopsy in the same patient, with both specimens showing loss of architecture and foci of necrosis consisting of nuclear dust, histiocytes, and scattered plasma cells without formation of granulomata or presence of multinucleated giant cells; these findings are classic for SLE lymphadenopathy. We believe this to be the first reported case of focal necrosis in the parotid gland directly associated with SLE.- - - - - - - - - - ranking = 14.06287084964keywords = gland (Clic here for more details about this article) |
3/50. Successful treatment of systemic lupus erythematosus and pulmonary hypertension with intravenous prostaglandin I2 followed by its oral analogue.Systemic lupus erythematosus (SLE) is sometimes reported to complicate fatal pulmonary hypertension. A 46-year-old woman, with a ten-year history of SLE and pulmonary hypertension, was admitted to our hospital complaining of dyspnea and chest pain. She suffered pulmonary hemorrhage and after steroid pulse therapy, she underwent continuous intravenous infusion of epoprostenol (prostaglandin I2) with corticosteroid for four weeks, which reduced the pulmonary artery pressure and resistance. Following the successful treatment, beraprost sodium, an oral PGI2 analogue, was given and it maintained pulmonary hypertension remittance for four years.- - - - - - - - - - ranking = 35.1571771241keywords = gland (Clic here for more details about this article) |
4/50. Immunoblastic sarcoma with features of sjogren's syndrome and systemic lupus erythematosus in a patient with immunoblastic lymphadenopathy.A patient with immunoblastic lymphadenopathy and features of sjogren's syndrome and systemic lupus erythematosus is presented. Clinical features included generalized lymphadenopathy, rash, alopecia, and synovitis, with associated laboratory abnormalities of a positive antinuclear factor and double-stranded dna antibodies, positive lupus band test, and hemolytic anemia. Symptoms of sjogren's syndrome included dry eyes and mouth and swollen parotid gland; biopsy results of the accessory salivary glands were positive. At autopsy immunoblastic sarcoma was found that involved the myocardium, which stained for both kappa and lambda light chains by immunoperoxidase techniques.- - - - - - - - - - ranking = 14.06287084964keywords = gland (Clic here for more details about this article) |
5/50. Systemic lupus erythematosus with polyglandular autoimmune syndrome type II: report of an unusual case.A patient with systemic lupus erythematosus (SLE) is described who had associated autoimmune thyroiditis with subclinical hypothyroidism and idiopathic Addison's disease. The presence of autoimmune thyroid disease and Addison's disease could classify her as having polyglandular autoimmune syndrome (PGAS) type II, which is the commonest of the three types of this syndrome. However, the additional presence of SLE in PGAS type II has not been described earlier. This patient appears to be the first such case.- - - - - - - - - - ranking = 35.1571771241keywords = gland (Clic here for more details about this article) |
6/50. Rosai-Dorfman disease of the parotid gland: cytologic and histopathologic findings with immunohistochemical correlation.Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.- - - - - - - - - - ranking = 42.188612548921keywords = gland (Clic here for more details about this article) |
7/50. 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 = lobe (Clic here for more details about this article) |
8/50. Fibrothorax and severe lung restriction secondary to lupus pleuritis and its successful treatment by pleurectomy.Pleural disease is a common pulmonary manifestation of systemic lupus erythematosus (SLE) that usually responds to corticosteroids and other immunosuppressive agents. In the present report, a new approach, pleural decortication, was used in a patient with medically refractory chronic pleuritis secondary to severe SLE. A 26-year-old woman with known SLE developed progressive dyspnea and pleuritic chest pain over several months. The other systemic manifestations of her lupus were controlled with cyclophosphamide and prednisone. A computed tomography scan revealed a persistent, small, loculated right pleural effusion; pleural thickening; and atelectasis of the right middle and lower lobes. Pulmonary function tests showed a severe restrictive defect. The patient was disabled by her severe dyspnea despite maximal medical therapy, and, therefore, surgery was considered. A right thoracotomy revealed entrapment of the right lung by dense visceral pleura. Decortication was performed. On pathology, pleuritis with vascular pleural adhesions was found. No lupus pneumonitis was noted. Postoperatively, a significant clinical improvement in dyspnea was evident within several weeks. On a 6 min walk test, the patient achieved 384 m with a Borg dyspnea scale rating of 2 compared with 220 m and a Borg dyspnea scale rating of 4 preoperatively. Her forced vital capacity improved from 24% predicted to 47% predicted, and her total lung capacity improved from 35% predicted to 54% predicted. Medical therapy of systemic lupus erythematosus has been proven to be effective in controlling pleuritis in most cases. However, in the event of refractory pleuritis or pleural thickening, decortication may be a viable alternative.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
9/50. Ring-enhanced mass in the brain of a woman with systemic lupus erythematosus and elevated serum CA19-9 level: brain abscess or metastatic tumor?--case report.A 70-year-old woman with systemic lupus erythematosus presented with a brain abscess manifesting as progressive monoparesis of the right lower extremity over 4 days. She had had no episodes of fever, and did not complain of headache or exhibit any signs of meningeal irritability. Computed tomography of the brain showed a round, low-density mass with strong ring enhancement in the left frontal lobe. Laboratory examination found a moderately elevated serum level of CA19-9, a marker of some digestive organ cancers. Together with the absence of febrile episodes, headache, and a rise in leukocyte count, the initial suspicion was metastatic brain tumor rather than brain abscess. However, diffusion-weighted magnetic resonance imaging depicted the mass as a very hyperintense area. The neuroimaging diagnosis was brain abscess. After conservative treatment with intravenous antibiotics for 6 weeks, the brain abscess completely resolved, and the patient was discharged without neurological deficits.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
10/50. Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis.Acute lupus pneumonitis was the presenting manifestation of systemic lupus erythematosus in six of 12 cases in this series. The clinical picture was characterized by severe dyspnea, tachypnea, fever and arterial hypoxemia. Radiographic findings included an acinar filling pattern which was invariably found in the lower lobes and was bilateral in 10 of the cases. Studies failed to reveal evidence of infection as a cause of the acute pulmonary infiltrates. All patients were treated with oxygen and corticosteroids; seven received azathioprine. Six patients survived and are clinically well 14 months to four years following their acute illness. Three of these patients have residual interstitial infiltrates with persistent pulmonary function test abnormalities indicating progression to chronic interstitial pneumonitis. Histologic sections of the lungs available from four patients revealed hyaline membranes and interstitial edema (four cases), acute alveolitis (two cases), arteriolar thrombosis (one case) and a prominent lymphocytic interstitial pneumonitis with organizing bronchiolitis (one case).- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
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