Hepatitis: Guillevin L

Guillevin L. · No affiliation provided · Nephrol Dial Transplant. · Pubmed #10489209 links to  free full text

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Guillevin L. · No affiliation provided · Rheumatology (Oxford). · Pubmed #10461469 links to  free full text

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Belizna CC, Hamidou MA, Levesque H, Guillevin L, Shoenfeld Y. · Internal Medicine Department, CHU Rouen, Rouen, France. · Rheumatology (Oxford). · Pubmed #19258377 No free full text.

Abstract: Vasculitis may be associated with infection, immunization or anti-microbial drugs. Infections are responsible for a number of different types of vasculitis. Conversely, patients with vasculitis may develop infections, which sometimes mimic relapse. The aim of this review is to summarize the various aspects of the inter-relationship between vasculitis and infection, and the physiopathological mechanisms involved, in light of our current knowledge from animal models. Currently, a causal relationship between infection and vasculitis has only been established in a few instances and many mechanisms remain hypothetical. This inter-relationship is further assessed from the point of view of clinical presentation and therapeutic options, based on case reports and prospective observational data.

Guillevin L, Pagnoux C. · Service de médecine interne, centre de référence maladies rares vascularites et sclérodermies systémiques, hôpital Cochin, Université Paris-V-René-Descartes, Paris. · Rev Prat. · Pubmed #18524111 No free full text.

Abstract: The treatment of necrotizing vasculitides may be based on several drugs, depending on the disease itself, its severity, its etiology and the expected prognosis. In practice, the least severe forms, associated with a prognostic score of 0, may be treated with corticosteroids only, while the ones associated with severity factors should be treated with an association of corticosteroids and immunosuppressants. The first-line treatment of viral-associated vasculitides should be based on antiviral drugs. The treatment duration of non-infectious vasculitides is typically 18 to 24 months. The improvement in prognosis is due to the treatment as well as to anti-infectious prophylaxis and careful management of potential complications.

Guillevin L. · Service de Médecine Interne, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris René Descartes, 27 rue du Faubourg Saint-Jacques, Paris, France. · Clin Rev Allergy Immunol. · Pubmed #18181034 No free full text.

Abstract: Necrotizing systemic vasculitides are severe diseases in which prognosis improved since steroids and cytotoxic agents have been prescribed. The outcome is now better, and remission is obtained in more than 80% of patients. In this review paper, we will cover the indications of conventional treatments and new biotherapies and other therapies. The strategies that improve patient's outcome are discussed in the light of the prospective trials organized by international research groups.

Guillevin L, Pagnoux C. · Department of Internal Medicine, Referral Center for Rare Diseases, Vasculitis and Scleroderma, Hôpital Cochin, AP-HP, Université de Paris René-Descartes, 27, rue du Faubourg Saint-Jacques, Paris, France. · Transfus Apher Sci. · Pubmed #17499762 No free full text.

Abstract: Plasma exchanges (PE) are a component of regimens prescribed to treat systemic necrotizing vasculidities. They are also part of the best therapeutic strategy for virus-induced vasculidities. The combination of antiviral agents and PE has proven efficacy against polyarteritis nordosa. This strategy is also effective for human immunodeficiency virus-associated vasculitis and, unlike cytotoxic agents, does not jeopardize the outcome of acquired immunodeficiency syndrome. Concerning the vasculitis seen in the context of hepatitis C virus-related cryoglobulinemia, PE contribute to better outcomes but, because of the poor efficacies of antiviral drugs, only about half of the patients achieve definitive recovery and relapses are frequent. The use of PE to treat antineutrophil cytoplasm antibody-associated vasculidities with severe renal insufficiency leads to improved renal function and thus fewer patients require dialysis. Although PE does not improve survival, their adjunction to corticosteroids and immunosuppressants for patients with alveolar hemorrhage could also limit the severity of this severe manifestation.

Guillevin L, Pagnoux C. · Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris-René-Descartes, Paris, France. · Allergol Int. · Pubmed #17460440 No free full text.

Abstract: Treatments of vasculitides have progressed markedly over the past few decades. The first attempts to obtain better-adapted therapeutic strategies evaluated the indications of conventional drugs, and their abilities prolong survival and reduce the number of relapses, while decreasing the severity and number of side effects. Many prospective clinical trials were organized by the French Vasculitis Study Group and the European Vasculitis Study group, and have contributed to optimizing targeted treatment strategies. Recent therapeutic strategies include immunomodulating methods, like plasma exchanges, or products, like intravenous immunoglobulins, or, more recently, new agents called biotherapies. Some of them have achieved promising positive effects, for example, anti-CD20 monoclonal antibodies, and are now being evaluated in prospective trials.

Guillevin L, Pagnoux C, Guilpain P, Bienvenu B, Martinez V, Mouthon L. · Department of Internal Medicine, French Vasculitis Study Group, Centre de Référence Vascularites et Sclérodermie, Hôpital Cochin, Université Paris Descartes, Paris, France. · Clin Rev Allergy Immunol. · Pubmed #17426364 No free full text.

Abstract: Biotherapy now holds a specific place in the therapeutic armamentarium for systemic vasculitides. Such therapy includes cytokines, such as (pegylated) alpha-interferon for hepatitis B virus-related polyarteritis nodosa and hepatitis C virus-related cryoglobulinemic vasculitis, and polyvalent immunoglobulin (IVIg), with well-defined indications and pending positive results. More specifically targeted monoclonal antibodies include antitumor necrosis factor-alpha or anti-CD20 for antineutrophil cytoplasmic antibody-associated vasculitides or anti-interleukin-5 and anti-IgE for Churg-Strauss syndrome. However, the exact indications of these latter new agents, as well as their optimal dosage and duration, are not defined. Therefore, they are prescribed mainly for patients with disease refractory to conventional therapy, in whom results are promising. Results of international ongoing trials will determine whether the agents may also have a place as first-line treatment.

Pagnoux C, Cohen P, Guillevin L. · Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France. · Clin Exp Rheumatol. · Pubmed #16859600 No free full text.

Abstract: Many viruses can be responsible for systemic vasculitis, the most frequent being hepatitis B virus-related polyarteritis nodosa (HBV-PAN), even though its incidence has decreased over the past few decades. Mixed cryoglobulinemia has been shown to be associated with hepatitis C virus (HCV) infection in more than 80% of the patients, but it remains asymptomatic in most of them with only a minority developing vasculitis. Human immunodeficiency virus (HIV), erythrovirus B19, cytomegalovirus, varicella-zoster virus and human T-cell lymphotropic virus (HTLV)-1 have also been reported to be associated with or implicated in the development of vasculitides. On the other hand, some bacteria, fungi or parasites can also cause vasculitis, mainly by direct invasion of blood vessels or septic embolization, leading, e.g., to the well-known feature of 'mycotic aneurysm'. Syphilitic aortitis and/or cerebrovascular disease and rickettsial diseases are other, more specific, bacteria-induced vasculitides. Recognizing an infectious origin of vasculitides is of great importance because treatment strategies differ from those applied to non-infectious forms. Effective antimicrobial drugs are mandatory to treat bacterial, parasitic or fungal infections, while the combination of antiviral agents and plasma exchanges has been proven to be effective against HBV-PAN. This latter strategy might also be effective against HIV-associated vasculitis and, unlike cytotoxic agents, does not jeopardize the outcome of HIV-infected patients. In the context of HCV-related cryoglobulinemic vasculitis, antiviral drugs are necessary to achieve recovery, in combination with low-dose corticosteroids and/or rituximab. In the near future, newer antiviral agents will probably also have their place in the therapeutic armamentarium for these patients.

Guilpain P, Servettaz A, Tamby MC, Chanseaud Y, Le Guern V, Guillevin L, Mouthon L. · Université Paris-Descartes, Faculté de médecine, UPRES EA 1833, site Cochin, Paris. · Presse Med. · Pubmed #16225258 No free full text.

Abstract: The pathogenesis of different types of systemic vasculitis negative for antineutrophil cytoplasm antibodies (ANCA) and involving small or medium-sized vessels is not very well documented. During polyarteritis nodosa (PAN), which is related to hepatitis B virus (HBV) infection, as well as during cryoglobulinemic vasculitides, associated with hepatitis C virus (HCV), and probably during Henoch Schönlein purpura, histological lesions may result from the deposition of immune complexes formed from viral antigens and from antibodies responsible for the activation of the classic complement pathway and for recruitment of polymorphonuclear neutrophils. Two other mechanisms are discussed for other types of ANCA-negative systemic vasculitis: immune complex deposition and sheer stress at arterial bifurcation points. A bacterial superantigen is suspected in Kawasaki disease but remains unproved.

Cohen P, Guillevin L. · Service de médecine interne, Hôpital Avicenne, Bobigny (93). · Presse Med. · Pubmed #15615248 No free full text.

Abstract: VIRUSES, THE CAUSE OF VASCULITIS: Although the majority of systemic vasculitis are of unknown causes, the responsibility of a viral infection has been formally demonstrated in some of them and specific treatment can permanently cure them. Each virus incriminated accounts for a particular type of vasculitis. HEPATITIS B VIRAL INFECTION (HBV): Is the cause of polyarteritis nodosa in 36 to 50% of cases. The onset of the symptomatology is acute, usually within a few months following the infection; it is comparable to that observed in the absence of HBV infection. CRYOGLOBULINEMIA RELATED TO THE HEPATITIS C VIRUS (HCV): The clinical manifestations are those of systemic vasculitis with particular tropism for the skin (involvement generally inaugural and almost constant), peripheral nerves and the glomerula. They occur fairly late during the infection. VASCULITIS ASSOCIATED WITH HIV INFECTION: There is strong tropism for the peripheral (multi-neuritis) and central nervous system. During acute parvovirus B19 infection Vasculitis lesions have occasionally been reported following the viremic phase, generally limited to one or several flares of vascular purpura predominating on the lower limbs. FOLLOWING VARICELLA-HERPES ZOSTER INFECTION: Vasculitis occasionally develops in the form of a central neurological deficiency (locomotor deficiency with or without aphasia around one month after an ophthalmologic herpes zoster) or involving the retina or, more rarely, the skin or the kidneys. VASCULITIS ASSOCIATED WITH CYTOMEGALOVIRAL INFECTION: Predominantly observed in immunodepressed patients, vasculitis after CMV infection is diffuse and basically involving the digestive tube, notably the colon, the central nervous system and the skin. A RARE COMPLICATION OF AN HTLV1 INFECTION: Vasculitis of the retina often in the form of necrotic retinitis is often associated with spasmodic paraparessia. THERAPEUTIC STRATEGY: For many vasculitis of viral origin, corticosteroid and immunosuppressive treatments are only indicated in second intention following failure with antiviral agents and the combination of antivirals and plasma exchanges.

Guillevin L. · Department of Internal Medicine, Hôpital Cochin, AP-HP, Université René Descartes-Paris V, 27, rue du Faubourg Saint-Jacques, Paris, France. · Clin Dev Immunol. · Pubmed #15559368 links to  free full text

Abstract: The best therapeutic strategy in virus-induced vasculitides should take into account the etiology of the disease and be adapted to the pathogenesis. The combination of antiviral treatments and plasma exchanges has been proven effective in polyarteritis nodosa (PAN). In human immunodeficiency virus (HIV)-related vasculitis this strategy is also effective and does not jeopardize, like cytotoxic agents, the outcome of AIDS. In vasculitis related to HCV-associated cryoglobulinemia, plasma exchanges improve the outcome but the poor effectiveness of antiviral drugs is not able to favor, usually, a definite recovery of the patients and relapses are frequent.

Guillevin L, Cohen P. · Department of Internal Medicine, Hôpital Avicenne, AP-HP, Université Paris-Nord, 125 rue de Stalingrad, 93009 Bobigny Cedex, France. · Curr Rheumatol Rep. · Pubmed #11798984 No free full text.

Abstract: The best therapeutic strategy in virus-induced vasculitides should take into account the etiology of the disease and be adapted to the pathogenesis. The combination of antiviral treatments and plasma exchanges has been proven effective in polyarteritis nodosa. In HIV-related vasculitis, this strategy is effective and does not jeopardize the outcome of AIDS, as do cytotoxic agents. In vasculitis related to hepatitis C virus-associated cryoglobulinemia, plasma exchanges improve the outcome, but the poor effectiveness of antiviral drugs usually does not favor a definite recovery of the patients. Relapses are frequent.

Trepo C, Guillevin L. · Departement of Hépato-gastroentérology, Hôtel Dieu, 1 Place de l'hôpital, Lyon, Cedex 02, 69288, France. · J Autoimmun. · Pubmed #11334492 No free full text.

Abstract: Numerous extrahepatic manifestations have been reported in patients with both acute and chronic hepatitis B (arthralgias or arthritis, skin rashes, glomerulonephritis and neuritis), all of which are present in polyarteritis nodosa (PAN) which is the most unique and spectacular extrahepatic manifestation. In the 1970s, the frequency of PAN due to the hepatitis B (HBV) reached 30%. Immunization programs explain the decrease and it is now down to 7%. PAN usually occurs within 6 months of infection. Clinical manifestations reflect this most classic form of PAN, Hepatic manifestations including, ALT/AST elevations are mild and usually overlooked. Besides HBV, other viruses may be responsible for cases of vasculitides including PAN, HIV, Parvovirus B19, and EBV. Different pathogenic mechanisms have been identified but immune complexes are mainly thought to be responsible. In glomerulonephritis, detailed immunostaining and ultrastructural findings indicate that HBe antigen (Ag) is more likely to be the responsible antigen. In PAN, fewer reports are available and early studies with poorly defined antibodies need to be revisited. Interestingly almost all cases of HBV/PAN are associated with wild-type HBV infection, characterised by HBe antigenemia and high HBV replication, supporting the concept that lesions could result from the deposit of viral Ag/Ab complexes soluble in Ag excess, possibly involving HBe Ag. The recent observation of PAN cases associated with precore mutation which abrogates the formation of HBe Ag challenges this view. It may suggest that other, still undefined, circulating HBV-related Ag(s) distinct from HBe Ag could be involved. Remarkably, none of the HBV/PAN cases or glomerulonephritis exhibit antineutrophil cycoplasmic antibodies (ANCA) reactivity. Viral PAN can now be completely separated from other form of vasculitis mostly autoimmune in nature. Based on the efficacy of antiviral agents in chronic hepatitis B and of plasma exchanges in PAN we combined both therapies to treat HBV PAN. This was associated with swift recovery, even in the most severe forms. The perfect time correlation between inhibition of virus replication and resolution of all bioclinical signs suggest a direct pathogenic role of the virus possibly via immune complexes. Traditional immunosuppressive and steroid therapy should no longer be used for HBV PAN cases.

Guillevin L, Cohen P. · Service de Médecine Interne, Hôpital Avicenne, Université Paris-Nord, 125, route de Stalingrad, 93009 Bobigny, France. · Ann Med Interne (Paris). · Pubmed #10896970 No free full text.

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Guillevin L, Lhote F. · University of Paris, France. · Adv Nephrol Necker Hosp. · Pubmed #10561736 No free full text.

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Lhote F, Guillevin L. · Service de Médecine Interne, Hôpital Delafontaine, Saint-Denis, France. · Pathol Biol (Paris). · Pubmed #10214619 No free full text.

Abstract: Necrotizing vasculitis is a heterogeneous group of systemic diseases characterized by inflammation of blood vessel walls. There is current agreement that viral infections can play a central role in the pathophysiology of systemic vasculitides responsible for a broad spectrum of clinical patterns. The hepatitis B and C viruses (HBV and HCV), the parvovirus B19, the human immunodeficiency virus, the HTLV 1, and the cytomegalovirus are the viruses most often implicated. Only the HBV and HCV are associated with fairly well-defined clinical pictures. The conventional management of systemic vasculitis rests on administration of a corticosteroid with an immunosuppressive (usually cyclophosphamide). The decision to treat rests on the nature and severity of the vasculitis. In viral vasculitis, the goal of therapy is to treat both the systemic condition and its cause at the same time. In this situation, conventional immunosuppressive therapy promotes perpetuation of the viral infection, exposing the patient to chronic lesions and to relapses. Antiviral therapy has demonstrated clear evidence of efficacy in vasculitis related to the HBV and, to a lesser degree, the HCV. For the other viruses data, in the literature are scant.

Le Hello C, Cohen P, Bousser MG, Letellier P, Guillevin L. · Service de Médecine Interne, Hôpital Avicenne, Université Paris-Nord, Bobigny, France. · J Rheumatol. · Pubmed #9918261 No free full text.

Abstract: Recombinant hepatitis B vaccination is widely used and severe side effects are rare. We describe 3 cases of vasculitis occurring after such immunization that are thought to have been vaccine induced. Vasculitides are now recognized as possible severe adverse side effects of immunization.

Guillevin L, Mahr A, Cohen P, Larroche C, Queyrel V, Loustaud-Ratti V, Imbert B, Hausfater P, Roudier J, Bielefeld P, Petitjean P, Smadja D, Anonymous00104. · Centre Hospitalier Universitaire Avicenne, Bobigny, France. · Arthritis Rheum. · Pubmed #15188337 links to  free full text

Abstract: OBJECTIVE: To assess the efficacy and safety of lamivudine, an antiviral agent that strongly inhibits hepatitis B virus (HBV) DNA replication, combined with plasma exchanges after short-term corticosteroids for HBV-related polyartertitis nodosa (PAN). METHODS: Ten patients (8 men, 2 women, mean +/- SD age 50.4 +/- 14.4 years) with previously untreated HBV-related PAN were included in a multicenter, prospective, observational trial. Oral prednisone (1 mg/kg/day) was given for 1 week, then tapered and withdrawn within 1 week. Then, lamivudine (100 mg/day or less in the case of renal insufficiency) was started for a maximum of 6 months. Plasma exchanges were performed simultaneously and scheduled as follows: 3/week for 3 weeks, 2/week for 2 weeks, then 1/week until hepatitis B e antigen (HBeAg) to anti-HBe antibody (HBeAb) seroconversion was obtained or until 2-3 months of clinical recovery was sustained. The primary trial endpoint was clinical recovery from HBV-PAN at 6 months. The secondary endpoint was loss of detectable serum HBeAg and HBV DNA, and HBeAg to HBeAb seroconversion at 9 months. RESULTS: One death, attributed to catheter-related septicemia, was recorded. At 6 months, all 9 survivors had achieved clinical recovery and by 9 months, 6 of 9 (66%) had seroconverted. CONCLUSION: The strategy of short-term steroids followed by lamivudine and plasma exchanges effectively led to recovery from HBV-PAN. Because of its oral administration and good safety profile, lamivudine should henceforth be considered the antiviral agent of choice to treat HBV-related PAN.

Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B, Anonymous00151. · H pital Avicenne, Bobigny, France. · Arthritis Rheum. · Pubmed #11263782 links to  free full text

Abstract: OBJECTIVE: To determine the long-term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), to compare the long-term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae. METHODS: Data from PAN, MPA, and CSS patients (n = 278) who were enrolled between 1980 and 1993 were collected in 1996 and 1997 and analyzed. Two prognostic scoring systems, the Five-Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS), were used to evaluate all patients at the time of diagnosis. RESULTS: The mean (+/- SD) followup of the entire population was 88.3 +/- 51.9 months (range 3 days to 192 months). Of the 85 deaths recorded, at least 41 were due to progressive vasculitis or its consequences. Death rates reflected disease severity, as assessed by the FFS (P = 0.004) and the BVAS (P < 0.0002), and the 2 scores were correlated (r = 0.69). Relapses, rarer in hepatitis B virus (HBV)-related PAN (7.9%) than in MPA (34.5%) (P = 0.004), occurred in 56 patients (20.1%) and did not reflect disease severity. Survival curves were similar for the subpopulation of 215 patients with CSS, MPA, and non-HBV-related PAN who were given first-line corticosteroids (CS) with or without cyclophosphamide (CYC). However, CS with CYC therapy significantly prolonged survival for patients with FFS scores > or =2 (P = 0.041). Relapse rates were similar regardless of the treatment regimen; only patients treated with CS alone had uncontrolled disease. CYC was associated with a greater frequency of side effects (P < 0.00001). CONCLUSION: Rates of mortality due to PAN (related or unrelated to HBV), MPA, and CSS reflected disease severity and were higher than the mortality rate in the general population (P < 0.0004). Rates of relapse, more common in MPA than HBV-related PAN patients, did not reflect disease severity. Survival rates were better among the more severely ill patients who had received first-line CYC. Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response.

Ben m'rad M, Leclerc-Mercier S, Blanche P, Franck N, Rozenberg F, Fulla Y, Guesmi M, Rollot F, Dehoux M, Guillevin L, Moachon L. · Department of Internal Medicine, Reference Center for Autoimmune and Inflammatory Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Paris-Descartes University, Paris, France. · Medicine (Baltimore). · Pubmed #19440116 No free full text.

Abstract: Drug-induced hypersensitivity syndrome (DIHS), also called drug rash with eosinophilia and systemic symptoms (DRESS), is a severe reaction usually characterized by fever, rash, and multiorgan failure, occurring 1-8 weeks after drug introduction. It is an immune-mediated reaction involving macrophage and T-lymphocyte activation and cytokine release, although no consensus has been reached as to its etiology. The skin, hematopoietic system, and liver are frequently involved. DIHS can mimic severe sepsis, viral infection, adult-onset Still disease (AOSD), or lymphoproliferation.We describe 24 consecutive patients with DIHS who were hospitalized between September 2004 and March 2008. Criteria for inclusion in this observational study were suspected drug reaction, eosinophilia >or=500/microL and/or atypical lymphocytes, involvement of at least 2 organs (skin being 1 of them), with suggestive chronology and exclusion of other diagnoses. Our cohort of 12 women and 12 men had a median age of 49 years (range, 22-82 yr), and 11 had skin phototype V or VI. Patients with mild or no rash were immunocompromised (7/24)- defined as treatment with prednisone (>or=10 mg/d) and another immunosuppressant drug, or human immunodeficiency virus infection. All patients were febrile (>38 degrees C), 14 had localized or generalized edema, 7 had pharyngitis, 8 had lymphadenopathy, 22 had hepatitis, 4 had nephritis, 2 had noninfectious and nonlithiasic angiocholitis or cholecystitis. Ten patients were hypotensive, 5 of whom had associated laboratory signs and/or imaging findings suggestive of acute myocardial dysfunction. Half of the patients had hemogram abnormalities, including eosinophilia. Nine DIHS patients fulfilled the Fautrel criteria for AOSD diagnosis, including glycosylated ferritin <20% in 4/11, with or without laboratory characteristics of hemophagocytosis. Twenty DIHS episodes occurred during the less sunny months of October to March.We determined 25-hydroxyvitamin D3 (25[OH]D3) levels in 18 patients and found that 9 patients had vitamin D deficiency (<25 nmol/L or <10 microg/L) and 5 had vitamin D insufficiency (25-50 nmol/L). Moreover, 25(OH)D3 levels were inversely correlated with ferritin values. After culprit-drug withdrawal, outcomes were favorable for all patients, including those with cardiac abnormalities under slow tapering of glucocorticoids.We recommend looking for the frequent but underdiagnosed hypersensitivity myocarditis with noninvasive diagnostic tools, such as N-terminal probrain natriuretic peptide, and promptly withdrawing the culprit drug and starting glucocorticoids. Vitamin D deficiency might be a DIHS risk or severity factor, especially for patients with high skin phototype and during the winter. Because DIHS clinical and laboratory patterns share similarities with AOSD and hemophagocytosis, DIHS should be included in their differential diagnoses.

Kluger N, Pagnoux C, Guillevin L, Francès C, Anonymous00011. · Service de Dermatologie, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Pierre et Marie Curie, Hôpital Tenon, 4 Rue de la Chine, F-75020 Paris, France. · Br J Dermatol. · Pubmed #18647311 No free full text.

Abstract: BACKGROUND: The cutaneous manifestations of microscopic polyangiitis (MPA) and polyarteritis nodosa (PAN) have not been compared since their distinction. Objectives To compare the clinical and pathological cutaneous manifestations in a series of patients with systemic MPA and PAN. METHODS: Patients with MPA (n = 162) and PAN (n = 248) from the database of the French Vasculitis Study Group were diagnosed according to the American College of Rheumatology and/or the Chapel Hill Consensus criteria. Purpura, livedo, nodules, urticaria, skin necrosis, oral and genital ulcers were recorded when present. Fifty-five skin biopsies were analysed. Clinical and histological skin data were compared in the following groups: MPA, PAN and two PAN subsets: PAN with and PAN without hepatitis B infection. The prevalence of systemic and biological manifestations were analysed in relation to the presence or absence of skin lesions. The chi(2) test was used for statistical studies. RESULTS: Cutaneous manifestations were present in 44% of MPA and PAN. Purpura was the most frequent manifestation (26% cases of MPA vs. 19% cases of PAN, P = 0.026). Urticaria was more frequent during PAN (6% vs. 1.2%, P = 0.015). Skin lesions were more frequent during PAN in the absence of HBV infection (54% vs. 30%, P < 0.05). No significant difference was detected from the histological data. Patients with skin lesions (either MPA or PAN) presented arthralgias and ocular manifestations more frequently. Mononeuritis multiplex was associated with skin lesions in the MPA group (P < 0.05). CONCLUSIONS: The clinical or histological analysis of cutaneous lesions is not helpful for distinguishing PAN from MPA.

Charlier C, Henegar C, Launay O, Pagnoux C, Berezné A, Bienvenu B, Cohen P, Mouthon L, Guillevin L. · Department of Internal Medicine, Hôpital Cochin, Université Paris Descartes, Faculté de Medicine, UPRES EA 4058, Assistance Publique-Hôpitaux de Paris, Paris Cedex 14, France. · Ann Rheum Dis. · Pubmed #18504289 No free full text.

Abstract: OBJECTIVE: To characterise major infectious complications and analyse potential risk factors in patients with Wegener granulomatosis (WG). METHODS: Data from 113 patients with WG (69 male) followed at least once between January 1984 and March 2006 in our internal medicine department, were analysed retrospectively. RESULTS: A total of 35 patients (mean (SD) age at WG diagnosis: 50.2 (13.05) years) developed 53 major infections. Infections were: bronchopneumonias (n = 19), herpes zoster recurrences (n = 9), cellulitis (n = 4), prostatitis (n = 4), spondylodiscitis and septic arthritis (n = 3), digestive tract infections (n = 2), Enterococcus faecalis or Staphylococcus aureus septicaemia (n = 2), viral hepatitis B reactivations (n = 2), post transfusion HIV infection with fatal cerebral toxoplasmosis, oesophageal candidiasis, disseminated herpes simplex and cytomegalovirus infection, cytomegalovirus retinitis, herpetic keratitis, herpetic stomatitis, Serratia sp. node suppuration and fever resolving under broad spectrum antibiotics (n = 1 each). Half of the major infectious episodes occurred within 3 years after WG diagnosis. Eight (7%) patients died, with two (2%) infection-related deaths. Patients diagnosed with WG before 1996 had a significantly higher rate of infection than those diagnosed later (48% vs 24%, p = 0.02). Cyclophosphamide and corticosteroids were independently associated with significantly higher risk of major infection (p<0.05 and <0.001, respectively). All patients treated since 1993 received antipneumocystosis prophylaxis. CONCLUSION: Cyclophosphamide and corticosteroids were associated with higher risk of infection. Despite systematic cotrimoxazole prophylaxis, major infections, mostly bronchopneumonias and herpes zoster recurrences, were still common in the course of WG.

Henegar C, Pagnoux C, Puéchal X, Zucker JD, Bar-Hen A, Le Guern V, Saba M, Bagnères D, Meyer O, Guillevin L, Anonymous00206. · Hôpital Cochin, Paris 5-René Descartes University, Assistance Publique Hôpitaux de Paris, France. · Arthritis Rheum. · Pubmed #18438816 links to  free full text

Abstract: OBJECTIVE: To establish a set of clinical and paraclinical criteria potentially useful as a diagnostic screening tool for polyarteritis nodosa (PAN). METHODS: The abilities of individual descriptive items to predict a diagnosis of PAN were evaluated by screening available data from 949 patients from the French Vasculitis Study Group database, including 262 with PAN and 687 with control vasculitides. Selected items were tested in a logistic regression model to establish a minimal set of nonredundant PAN-predictive criteria. The discriminative accuracy of these items and of the American College of Rheumatology (ACR) 1990 criteria were assessed by reapplying them to the initial patient sample and a subgroup restricted to PAN and microscopic polyangiitis (MPA) patients. A computer simulation procedure was conducted on artificially generated patient data to evaluate the usefulness of these criteria in predicting a diagnosis of PAN. RESULTS: The analysis resulted in the retention of 3 positive predictive parameters (hepatitis B virus antigen and/or DNA in serum, arteriographic anomalies, and mononeuropathy or polyneuropathy) and 5 negative predictive parameters (indirect immunofluorescence detection of antineutrophil cytoplasmic antibody; asthma; ear, nose, and throat signs; glomerulopathy; and cryoglobulinemia) for the criteria set. These criteria yielded 70.6% sensitivity for all control vasculitides and 89.7% for MPA controls, with 92.3% specificity for all controls and 83.1% for MPA controls. The discriminant abilities of this set of items outperformed the ACR 1990 criteria in all analytical situations, showing better robustness to variations in the prevalence of individual vasculitides. CONCLUSION: The use of positive and negative discriminant criteria could constitute a sound basis for developing a diagnostic tool for PAN to be used by clinicians. Further prospective analyses and validations in different populations are needed to confirm these items as satisfactory diagnostic criteria.

Guillevin L. · Service de médecine interne, centre de référence des maladies rares, vascularites et sclérodermies, hôpital Cochin, AP-HOP, université Paris-Descartes, 27 rue du Faubourg-Saint-Jacques, 75014 Paris, France. · Rev Med Interne. · Pubmed #18029060 No free full text.

This publication has no abstract.