Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003864 (arthritis)
69,039 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The coexistence of systemic lupus erythematosus (SLE) and thymoma is rare. We describe 2 female patients with this combination. A 48-year-old woman presented with dyspnea due to a left pleural effusion. Her past history revealed over the previous 3 years the development of anemia, thrombocytopenia, alopecia, pericardial effusion and proteinuria. Four months prior to this hospitalization, the patient was first admitted due to purpura. At that time, laboratory tests revealed an elevated sedimentation rate, elevated titers of ANA and anti-DNA. Chest X-ray demonstrated a widened mediastinum, and upon operation an encapsulated thymoma was excised. Four months following the thymectomy, the patient is unresponsive despite high dose steroid therapy. Another patient, a 30-year-old woman, presented with SLE (cutaneous, arthritis, anemia, positive ANA and high titers of anti-DNA) and thymoma simultaneously. Six years after thymectomy the patient is in SLE remission. Thymectomy in mice prone to autoimmunity (NZB/W mice) has been shown to accelerate the autoimmune manifestations. Conversely, the opposite effect is seen in MRL/lpr mice. The immunological effect of adult thymectomy on the course of human SLE remains to be established, on a larger series of patients. It seems that the heterogenicity of human patients is exemplified by the contrasting effects of thymectomy for thymoma in SLE patients.
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PMID:Systemic lupus erythematosus and thymoma--a double-edged sword. 764 92

Juvenile chronic arthritis is a heterogenous disease with an ill-defined pathogenesis. In our study, synovial fluid (SF) and peripheral blood (PB) of 70 children with oligoarthritis were investigated; bacteria-specific lymphocyte proliferation and antibodies to arthritogenic bacteria were determined. Specific cellular immune responses in SF but not in PB were found in 4/7 patients with either Lyme- or reactive arthritis (60%). In comparison, in subgroup JCA II (n = 45) encompassing mainly elder HLA B27 positive boys, a specific response in SF but again not in PB was detected in 10 children to Yersinia enterocolitica (YE), in four children either to Borrelia burgdorferi (BB) and Chlamydia trachomatis (CT), and in one child to Campylobacter jejuni (CJ). In contrast, in subgroup JCA I (n = 17) encompassing mainly young ANA-positive girls with chronic iridocyclitis, no specific response was found. The correlation of the synovial cellular and the humoral immune responses was 100% in the case of BB and 50% for YE; no antibodies against CT or CJ were detectable. Neither specific cellular nor humoral immune responses were detected against Salmonella or Shigella. We conclude that, in the pathogenesis of some patients with JCA, bacterial microbes have a triggering role. Mainly YE, but also BB and CT are responsible for cases of JCA in which no symptomatic infection preceded.
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PMID:[Synovial cellular immune response to bacterial pathogens in patients with chronic juvenile arthritis]. 769 86

In order to adequately care for patients with chronic disorders and to properly allocate resources, the epidemiology of the underlying disease must be know. Proper population based studies involve substantial planning and educational programs, however. To prepare for such a study of pediatric rheumatic disorders, we performed a referral-based pilot study. During an eleven-month period pediatricians all over Costa Rica were asked to refer to us all new cases of possible rheumatic disorders among children less than 16 years of age, using the EULAR criteria for juvenile chronic arthritis. The children were evaluated at the National Children's Hospital. An annual incidence of 5.9 cases of all types of pediatric rheumatic diseases per 100,000 was found. Incidences of 5.4 per 100,000 children below 16 years of age and 6.1 for children below 12 years of age were observed for juvenile chronic arthritis (JCA). 77% of the JCA cases were of pauciarticular onset, and 23% were of polyarticular onset. No cases of systemic JCA were diagnosed. The female to male ratio was 3:2. Antinuclear antibodies were positive in 13% of the JCA cases, and IgM rheumatoid factor was found in 15% of the children. Chronic iritis was noted in only 2 cases; both were girls and both were ANA positive. The incidence found was low compared to population-based studies, but in the same range as hospital-based investigations.
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PMID:Juvenile chronic arthritis in Costa Rica. A pilot referral study. 777 91

Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F = 1:4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, 1 due to cardiac and 2 due to central nervous system involvement.
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PMID:Childhood systemic lupus erythematosus in south India. 795 96

Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynaud's phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.
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PMID:Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis: treatment with high dose prednisone. 808 81

In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.
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PMID:Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. 847 24

Epidemiological, clinical and serological data of uranium miners with symptoms of connective tissue diseases (CTD) were collected during the control examinations for occupational lung diseases since 1975. Twenty eight definite (four or more ARA criteria) and 15 probable (2-3 ARA criteria) SLE were diagnosed. The estimated prevalence among heavily silica exposed uranium miners was up to 93 in 100,000. The only significant differences to nonexposed SLE patients were decreased frequency of arthritis and photosensitivity and the absence of anti-Sm and anti-U1-RNP antibodies. ANA were found in all definite SLE patients examined with the following specificities: anti-dsDNA (in 44.4%), & anti-Ro/SSA (in 55.6%, four cases together with anti-dsDNA) and anti-La/SSB (in 22.2%). The autoantibody profiles of patients with probable SLE were similar, but with a lower frequency of ANA, anti-dsDNA and anti-Ro/SSA. Middle to high-titred autoantibodies to dsDNA, Ro/SSA and La/SSB were detected in 3.2% uranium miners with no (N = 1229) and in 20.6% with some symptoms (one ARA criterion and/or two or more of other CTD typical symptoms, N = 68) of CTD development. We conclude, that the strong exposure to dust with a high content of silica may predispose to or initiate the development of SLE. The detection of SLE-typical antibodies in quartz dust-exposed miners may indicate a higher risk for the development of systemic autoimmune disease.
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PMID:Systemic lupus erythematosus after heavy exposure to quartz dust in uranium mines: clinical and serological characteristics. 864 29

Antineutrophil cytoplasmic antibodies (ANCA) are important serological markers for the primary systemic vasculitides, including microscopic polyarteritis and necrotizing crescentic glomerulonephritis. Numerous reports have established the clinical utility of ANCA titer in monitoring disease activity, relapses, and response to treatment. ANCA, detected by indirect immunofluorescence (IIF) assays using patient's serum and ethanol-fixed human neutrophils, produce two common fluorescent staining patterns: cytoplasmic (C-ANCA), involving a 29-kD neutral serine protease termed proteinase 3 (PR3), and perinuclear (P-ANCA), the result mainly of myeloperoxidase (MPO), but occasionally by other components of the azurophilic granules including lysozyme, elastase, cathepsins, and lactoferrin. Some sera contain granulocyte-specific antinuclear antibodies (GS-ANA), which require formaldehyde fixation of neutrophils to cross link cytoplasmic antigens for distinguishing between ANCA and the GS-ANA by IIF. Positive IIF is confirmed by Western blot analysis or specific enzyme-linked immunosorbent assay for PR3, MPO, and other neutrophil granule antigens. The C-ANCA pattern is highly specific for Wegener's granulomatosis, a disease characterized by granulomatous inflammation, necrotizing and crescentic glomerulonephritis, and vasculitis; P-ANCA is found in sera of individuals with vasculitis, glomerulonephritis, and several other diseases. ANCA are predominantly immunoglobulin (Ig)G isotype, but may be IgM and IgA. Various pathophysiologic mechanisms have been proposed involving ANCA-mediated neutrophil activation in a hypothetical model of vasculitic diseases: positive signals via the FcgammaRII (CD32) receptor after IgG-ANCA binding to membrane-associated PR3, relevant cytokines, production of adhesion molecules on both activated neutrophils and endothelial cells, and the release of neutrophil reactive oxygen species and degranulation causing endothelial cell damage. Interference of C-ANCA with PR3 proteolysis and PR3 inhibition physiologically by the alpha1-proteinase inhibitor may have a pathogenic role. No convincing data have been reported for the existence of autoreactive T lymphocytes reactive to any degree with the neutrophil azurophilic enzymes. Studies of various drug- and infectious agent-related diseases and ANCA may contribute to understanding the mechanism(s) involved in some vasculitides.
Semin Arthritis Rheum 1995 Dec
PMID:Antineutrophil cytoplasmic antibodies: major autoantigens, pathophysiology, and disease associations. 865 May 85

A 20-year old female patient with systemic lupus erythematosus with butterfly-erythema, arthritis, high positive ANA and anti-ds-DNA titres has to have laparatomy because of an acute abdomen. Histological diagnosis is a necrotizing vasculitis of the appendix. In systemic lupus erythematosus an intestinal vasculitis can be found which might be as severe as in polyarteritis nodosa.
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PMID:[Necrotizing vasculitis of the appendix and cecum in systemic lupus erythematosus]. 896 88

In this article, a girl with an especially severe form of autoimmune acute hepatitis was successfully treated with immunosuppressive medication. This 11 year-old girl was hospitalized with the chief complaint of persistent jaundice for three months. The pathohistology of liver biopsy was chiefly composed of massive necrosis and heavy lymphoid infiltration. With the clinical findings of pericardial effusion, arthritis, positive ANA, and positive anti-dsDNA, she initially was mistaken for a case of unusual SLE complicated with hepatic involvement. Differentiating the autoimmune hepatitis from the hepatic involvement of SLE for this case is illustrated by a review of the pertinent literature and our experiences with SLE. The characteristic features of autoimmune hepatitis, the relatively low titer of anti-dsDNA, and the inherent low level of C4 finally led to the conclusion that the cause for her liver disease was in favor of autoimmune hepatitis.
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PMID:Lupus-like autoimmune acute hepatitis: report of one case. 907 87


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