[Panarteritis nodosa. Diagnosis, course].
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Biomedical subjects
Publications and source records attributed to L Guillevin.
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Human polyvalent immunoglobulins administered intravenously have shown to be effective in some immune diseases. We administered high dose-IV Ig (2g/kg/session) in five patients with severe chronic systemic diseases. Their condition did not improve, except in one case where a transient response was noticed. Considering the inconsistent results and high cost of IV Ig, double blind studies are required to determine the conditions in which high dose IV Ig may be more effective than conventional treatments.
Ten patients with severe systemic diseases, including systemic lupus erythematosus (n = 2), polymyositis (n = 2), essential mixed cryoglobulinaemia (n = 2), rheumatoid arthritis vasculitis (n = 3) and Wegener's granulomatosis (n = 1), were treated with 3 consecutive plasma exchanges synchronized with pulse cyclophosphamide. This therapeutic regimen was applied every 4 weeks initially and thereafter every 6 weeks in case of positive response after the first 3 cycles; it was combined in all patients with corticosteroid therapy. The treatment was administered for severe flare-up of the disease in 7 patients and for failure of previous treatments, including corticosteroids, cyclophosphamide and plasmapheresis, in 3 patients. Three kinds of response were observed: lasting complete remission without relapse after synchronization had ceased in 4 patients, partial clinical remission with post-synchronization relapse in 5 patients, and primary failure without any clinical response to treatment in only 1 patient. These results suggest that repeated plasma exchanges synchronized with cyclophosphamide are effective against progressive autoimmune diseases and in cases where conventionally administered immunosuppressive treatments had failed. However, this type of treatment cannot prevent long-term relapses, and only a prospective study can evaluate its success in terms of survival.
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OBJECTIVE: To delineate the different types of inflammatory vascular diseases (IVD) occurring in patients with human immunodeficiency virus (HIV) infection. METHODS: Muscle, nerve, or skin biopsy specimens from 148 symptomatic HIV-infected individuals were reviewed, and subgroups of vasculitis were identified using the American College of Rheumatology (ACR) 1990 clinicopathologic criteria for the classification of vasculitis. RESULTS: IVD was documented in 34 patients (23%) and included necrotizing arteritis (3 patients), non-necrotizing arteritis (1 patient), neutrophilic IVD (7 patients), mononuclear IVD (17 patients), and other small vessel inflammatory changes (6 patients). According to the ACR criteria, 11 patients could be classified as having a distinct category of vasculitis, including polyarteritis nodosa (4 patients), Henoch-Schönlein purpura (1 patient), and drug-induced hypersensitivity vasculitis (6 patients), and 23 were classified in the group "other vasculitis, type unspecified." One patient had hepatitis B virus surface antigenemia, 2 had cryoglobulinemia, and 2 were coinfected by human T lymphotropic virus type I. Cytomegalovirus inclusions and antigens were found in endothelial cells in 1 patient. HIV antigens and genome were detected in perivascular cells of 2 of the 3 patients with necrotizing arteritis; in 1, HIV-like particles were seen by electron microscopy. Immune deposits were found in small vessel walls in 5 skin biopsy samples showing small vessel vasculitis and in the muscle of the 3 patients with necrotizing arteritis. CONCLUSION: A wide range of inflammatory vascular diseases may occur in HIV-infected individuals. Vascular inflammation appears multifactorial and may result from HIV-induced immunologic abnormalities and exposure to a variety of xenoantigens, such as HIV itself, other infectious agents, and drugs.
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Essential mixed cryoglobulinemia is frequently associated with chronic hepatitis. This report presents four cases of cryoglobulinemia with vasculitis associated with chronic hepatitis related to hepatitis C virus infection. Hepatitis C virus infection was ascertained in the four patients by both the presence in the serum of anti-HCV antibodies detected by the four-antigen recombinant immunoblot assay and of HCV RNA detected by polymerase chain reaction. In two patients tested, anti-HCV antibodies were not detected after centrifugation in the purified cryoglobulin but were detected in the supernatant. HCV RNA was detected in the purified cryoglobulin in all four patients and was detected in the supernatant in three patients. In one patient receiving recombinant interferon alfa, serum aminotransferases normalized and cryoglobulin disappeared; in another patient receiving recombinant interferon alfa, serum aminotransferases remained high and the cryoglobulin persisted. The presence of HCV RNA in the cryoglobulin and the parallelism of the changes of the cryoglobulinemia and of the serum aminotransferases during recombinant interferon alfa administration suggest that HCV infection is responsible for the production of cryoglobulinemia and vasculitis. It is proposed that HCV infection is a cause of cryoglobulinemia associated with chronic hepatitis.
The study of 30 patients with systemic lupus and of 153 relatives failed to show any differences in HLA and TCR beta haplotype frequencies between patients and relatives. A significant interaction between TCR V beta and HLA-DR/DQ genes in the response to the peptide U1-RNP A 35-58 was demonstrated.
Stringent conditions have been applied to set up an "enzyme-linked immunosorbent assay" to detect anti-endothelial cell autoantibodies. These predominated in Sneddon's syndrome.
Twelve cases of Pneumocystis pneumonia in patients with Wegener's granulomatosis were compared with a control-group of 32 patients. Our study showed that the dose of cyclophosphamide received in the first three months and a profound lymphocytopenia were strongly predictive of a risk of Pneumocystis carinii pneumonia in these patients.
OBJECTIVE: treatment of HIV-related vasculitis, avoiding prolonged immunosuppressive therapy. DESIGN: prospective pilot study of HIV-related neurological vasculitis. PATIENTS: two HIV-infected patients with histologically proven vasculitis. INTERVENTION: short-term corticosteroid followed by zidovudine combined with plasmapheresis. MAIN OUTCOME MEASURES: clinical, biological, immunological and electromyographic evaluation. RESULTS: complete neurological recovery. CONCLUSION: excellent tolerance and efficacy of combined zidovudine and plasmapheresis therapy in peripheral neurological HIV-related vasculitis.
Of 53 documented cases of Yersinia enterocolitica septicemia reported to the French national registry between 1985 and 1991, 43 files contained sufficient information on antibiotic treatment to be analyzed retrospectively. All patients had at least two positive cultures of blood collected before the initiation of treatment. All strains were susceptible in vitro to the antibiotics that are usually active against gram-negative rods except for older beta-lactam agents (i.e., aminopenicillins and first-generation cephalosporins). No multiresistant strain was isolated. Only four (7.5%) of the 53 patients died. Aminopenicillins, first-generation cephalosporins, and--when prescribed alone--amoxicillin/clavulanate were not effective. Third-generation cephalosporins, most often used in combination with other antibiotics, were successful in 85% of cases. Fluoroquinolones--alone or in combination--cured all of 15 infections, with patients improving rapidly and becoming apyretic within 1-4 days. These agents therefore seem to constitute the best treatment.
Heart disease is a rare but important complication of polymyositis. Diagnosis of myocardial disease is usually based on non-specific clinical, electrocardiographic, and echocardiographic data. This paper reports a case of polymyositis with myocardial disease diagnosed by myocardial imaging with radiolabelled antibody to myosin, a specific marker of the necrotic myocardial fibre.
Five patients developed neurologic symptoms a few hours to 2 months after being stung by a non-hooking arthropod with immediate cutaneous reaction. The patients had no clinical or serologic evidence for Lyme borreliosis and rickettsial disease. Clinical and electrophysiologic findings were consistent with a mixed axonal and demyelinating mononeuropathy, a monomelic multiple mononeuropathy, a mononeuropathy multiplex, a radiculoneuritis, and a distal symmetric polyneuropathy. Muscle and nerve biopsies showed lymphoplasmacytic small-vessel vasculitis in all patients, and wallerian degeneration in three. These patients, and 17 others from the literature, indicate a spectrum of peripheral neuropathies occurring after insect and spider stings.
The authors study the possible involvement of retrovirus in Sjögren's syndrome. They report a preliminary study of 20 French and 13 Ecuadorian patients with a primary Sjögren syndrome. A previous epidemiologic study showed a HTLV1 seroprevalence going from 0.04 to 6% on different regions of Ecuador. The results of this study show the presence of anti-HTLV1 antibodies only in 2 Ecuadorian patients and does not allow a final conclusion of the role of HTLV1 virus. Nevertheless other studies continue on the serie using minor salivary gland specimens obtained by biopsy. Recent publications and the notion of an "endogen" retrovirus will may be lighten a new day in this research.
OBJECTIVE: Antibodies directed against components of neutrophil cytoplasm have been detected in various systemic vasculitides and especially in Wegener's granulomatosis. In polyarteritis nodosa (PAN) and Churg-Strauss syndrome, few data are available and correlation between clinical manifestations and antineutrophil cytoplasm antibodies (ANCA) has not been established. Therefore, we tested, before treatment of vasculitis, 62 consecutive patients suffering from PAN with hepatitis B virus (HBV) markers, PAN of unknown etiology or Churg-Strauss syndrome. METHODS: Only patients with PAN and Churg-Strauss syndrome were included in the study. The diseases were histologically and/or angiographically proven. Every patient's serum was tested by an indirect immunofluorescence assay (IFA) and, in 37 cases, by an enzyme linked immunosorbent assay (ELISA). RESULTS: ANCA detected by IFA were observed in 10.7% of the patients with PAN with HBV markers, in 27.3% of the patients with PAN without HBV markers and in 66.7% of the patients with Churg-Strauss syndrome. When ELISA was performed, 11.1% of the patients with PAN associated with HBV infection, 20% of the patients with PAN without HBV markers and 55.6% of the patients with Churg-Strauss syndrome were positive. ANCA were positively correlated with asthma and purpura and negatively correlated with HBV markers. CONCLUSION: Regardless of the technique used, Churg-Strauss syndrome was associated with ANCA in about 60% of the cases while, in PAN of unknown etiology, ANCA were found in about 25% of cases. In contrast, IFA and ELISA only detected ANCA in a limited number of cases of PAN related to HBV infection. ELISA positivity in patients with PAN and Churg-Strauss syndrome was usually associated with antimyeloperoxidase antibodies. In our cases of PAN, ANCA and purpura were significantly correlated, suggesting that, in these cases, small vessels are involved and therefore macroscopic and microscopic PAN coexist. Thus it seems that ANCA are essentially present in the cases of small vessel vasculitis, as has been described, and are not a marker of pure macroscopic PAN, at least at our present level of understanding of these antibodies.
Rapidly progressive glomerulonephritis (RPGN) is rarely associated with macroscopic polyarteritis nodosa (PAN), as seen in this series of 7 out of 235 patients. The clinical symptoms of PAN were as follows: myalgias 6; fever 1; arthralgias and mononeuropathy multiplex 5; cutaneous vasculitis 3; arterial hypertension 4, 3 of which were malignant. The mean proteinuria was 2.7 g/24 h; creatininemia 458 microM/l; microscopic hematuria was present in 4 of the 7 patients; only 1 patient was anuric. Markers of hepatitis B virus were absent in all cases. Arteriography revealed microaneurysms and renal infarcts in 6 patients and distal arterial stenosis in one. Renal biopsies from all 7 patients demonstrated extracapillary glomerulonephritis, which was associated with tubulointerstitial fibrosis in one. Necrotizing vasculitis lesions were associated in 5 cases. Immunofluorescence was positive in 5 cases. The association of RPGN and PAN exists and may be underestimated due to the lack of systematic angiographic examinations during RPGN and renal biopsies in PAN patients with renal involvement.