摘要:

There is limited evidence for the association of specific malignancies with rheumatoid arthritis (RA). Monoclonal gammopathies can occur in RA. Their predictive value for the development of a lymphoproliferative disorder remains unclear and disputed. We reviewed charts of 214 RA patients all of whom had at least one serum protein electrophoresis. We performed a retrospective study of 12 patients with RA and an M spike. We further characterized the M spike by serum immunofixation and bone marrow studies. The median age at which the M spike was identified was 69 years. IgG was the predominant gammopathy in 50% of patients, with no difference in the amount of kappa (&kgr;) and lambda (&lgr;) chains.One patient was diagnosed with multiple myeloma, one with an undefined primary lymphoproliferative disorder, one with T-cell leukemia, five with myelodysplastic syndrome, and four with monoclonal gammopathy of undetermined significance at most recent evaluation. Of our patients, 42% had a myelodysplastic syndrome, which has not been previously reported, and, in contrast to previous reports, no lymphomas were identified. The follow-up evaluation of patients with myelodysplastic syndrome and monoclonal gammopathy of undetermined significance is important because they may progress to an overt neoplasia.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Pulmonary hypertension is a well-recognized complication of connective tissue diseases (CTD), with clinical symptoms typically limited to those with relatively advanced disease. The objective of this study was to identify patients with early pulmonary vasculature changes using cardiopulmonary exercise testing with pulmonary artery catheterization. Four patients with CTD and normal Doppler echocardiogram results identified as high risk for pulmonary hypertension on the basis of disease process underwent cardiopulmonary exercise testing with pulmonary artery catheterization. All patients had normal mean pulmonary artery pressures at rest (<25 mmHg), but exhibited an abnormal rise in mean pulmonary artery pressure with exercise. Cardiopulmonary exercise testing should be considered in patients at high risk for pulmonary hypertension to identify those individuals who may benefit from early therapeutic intervention.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Some authors have found a strong statistical association of antibodies to prothrombin (aPT) with thrombosis in patients with antiphospholipid syndrome (APS); others have not confirmed this finding. It is unknown if the detection of aPT, in addition to anticardiolipin (aCL) and anti-&bgr;2-glycoprotein-I (a&bgr;2GP-I) antibodies, provides additional information in the clinical study of these patients. We studied 38 patients with primary antiphospholipid syndrome and 466 patients with systemic lupus erythematosus (SLE; 24 had a history of thrombosis and 69 had secondary APS). All were tested by ELISA for serum aPT (IgG and IgM) using irradiated and plain plates. We also detected aCL and anti-&bgr;2GP-I by ELISA. One hundred sera from clinically healthy individuals were used as controls.Twenty-six percent and 11% primary APS sera were positive for IgG and IgM aPT, respectively, compared with 3% and 5% of controls (p< 0.001, both comparisons). We found no difference in the frequency of aPT in SLE patients and controls, but aPT were positive in 46% of lupus patients with a history of thrombosis (20% IgG, 33% IgM) and in 9% of those without thrombosis (6% IgG, 5% IgM;p< 0.001, both comparisons). Likewise, there was a significant difference in the frequency of aPT in SLE patients with (22%) or without (9%) secondary APS (p< 0.001). aPT titers decreased two- to sixfold when tested in plain plates. Thirty-five of 38 primary APS sera (92%) had IgG anti-&bgr;2GP-I and 12 (31%) had aCL. No patient had aPT as the only antibody. The higher binding of aPT on irradiated plates suggests that aPT recognize a hidden epitope exposed by negative surfaces. The higher frequency of aPT found in patients with primary APS, SLE with thrombosis, or with secondary APS may suggest concerted pathogenic actions with other autoantibodies, but the detection of aPT does not seem to be of clinical value.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

The authors describe a case of idiopathic nodular panniculitis (Weber-Christian disease) with recurrent febrile episodes resistant to glucocorticosteroids and methotrexate (MTX) in various combinations with hydroxychloroquine, azathioprine, cyclosporine, colchicine, and doxycycline. Thalidomide at 100 mg/day has induced a remission for 3 years.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

We describe a patient with a chronic case of pulmonary involvement of Takayasu’s arteritis in the resected lung. A 49-year-old woman was first diagnosed with Takayasu’s arteritis at age 30 years. On her first admission, she presented with Takayasu’s arteritis and pneumonia with cavitation in the left lung. After recovering from pneumonia, she was treated initially with prednisolone, 30 mg/day, and remained well until she developed hemoptysis at age 34 years. Findings suggesting aspergilloma were found in the same lobe on chest x-ray film when she was 46 years of age. By age 49 years, the hemoptysis became massive, and she was admitted for surgery. Left upper lobectomy and partial resection of S6 and S8 pulmonary segments were performed. Histologic analysis of the resected lung revealed typical pathologic findings of pulmonary artery involvement in Takayasu’s arteritis, such as stenosis recanalization and a vessel-in-vessel feature, but not active vasculitis. Infection probably occurred in the cavity of the infarcted tissue. Pulmonary artery involvement is common in Takayasu’s arteritis, but the aspergilloma in this corticosteroid-treated patient is an uncommon complication.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Opportunistic infections are a theoretical concern with the use of tumor necrosis factor antagonists, as these agents can impair host immunity. A 61-year-old man with rheumatoid arthritis being treated with only etanercept (soluble tumor necrosis factor receptor) and in remission from the rheumatoid arthritis presented with dorsal tenosynovitis of his right wrist. The synovitis was not improved by a local corticosteroid injection. Surgical exploration showed granulomatous inflammation, and cultures were positive forMycobacterium marinuminfection. Etanercept was discontinued 4 months after the hand infection started. The infection resolved completely with surgery and antibiotic therapy with clarithromycin 500 mg twice daily orally for 3 months. AlthoughM. marinuminfection was locally invasive, there was no systemic dissemination of the infection despite ongoing etanercept therapy for 4 months before it was discontinued. With more frequent use of this class of agents for rheumatoid arthritis and Crohn’s disease, we caution physicians to be alert for the possibility of this kind of local opportunistic infection.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

There have been reported cases of children with histories of pauciarticular juvenile chronic arthritis (JCA) later developing myasthenia gravis (MG) as young adults. This is intriguing because it had been considered rare to diagnose a second autoimmune disease in a patient with pauciarticular JCA, unlike in those with adult-onset rheumatoid arthritis. We report a case of MG in a 20-year-old woman with a history of pauciarticular JCA. She presented with bilateral ptosis, weakness, and a history of dysphagia. The diagnosis was confirmed with positive serum acetylcholine receptor antibodies (2000 nm/L) and electromyography showing a decremental response to repetitive muscle stimulation. The patient’s inflammatory arthritis was quiescent at diagnosis. The patient underwent a surgical thymectomy and was treated with pyridostigmine, intravenous immunoglobulin, and corticosteroids with a fluctuating clinical course. Previous cases have been reported of MG associated with this subtype of JCA, suggesting a connection in autoimmune pathology. The earlier recognition and management of MG in a patient with pauciarticular JCA presenting with weakness may improve the prognosis of this disease.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Pyogenic aseptic arthritis, pyoderma gangrenosum, and cystic acne (PAPA) syndrome is an unusual triad that was recently mapped to a chromosome 15q mutation. We describe a patient from this kindred in whom hypogammaglobulinemia and elevated tumor necrosis factor-&agr; serum levels were detected. The patient responded well to intravenous gammaglobulin and intra-articular corticosteroid therapy. Immune abnormalities can be found in PAPA syndrome and could be the consequence of the chromosomal abnormalities affecting candidate genes on this chromosome with subsequent abnormalities in cytokine or chemokine secretion. Rheumatologists should be alert for this syndrome. Correction of the immune abnormalities may be effective in controlling the disease manifestations.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

We report a case of a 23-year-old man with adult onset Still’s disease (AOSD) developing a rare, life-threatening complication of thrombotic microangiopathy (TMA). While the AOSD was in an active phase, our patient first developed hemolytic uremia syndrome, soon followed by convulsions, sudden loss of vision, and thrombotic angiopathy of retinal vessels. After immediate and aggressive treatment with high dose prednisolone and 18 courses of plasmapheresis, he recovered from this severe complication. We think that the occurrence of TMA in AOSD may not be coincidental, although more reports are needed to support this. Early recognition and aggressive immunotherapy can allow patients with AOSD to completely recover from this life-threatening complication.

ISSN:1076-1608    

出版商:OVID    

年代:2002

数据来源: OVID