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PubMed1980

Left atrial myxoma presenting as a systemic vasculitis.

Left atrial myxoma has often stimulated systemic vasculitis or a connective tissue disease, but the diagnosis is usually suggested by cardiac symptoms or signs. The present case report is both unusual and instructive in that the patient presented with a painless left foot drop followed by digital vasculitis. Laboratory findings included depressed total hemolytic complement and elevated anti-double-stranded DNA. Despite frequent and careful observation, a heart murmur was not appreciated. Because of the unusual features and the patient's frequent military travels, the diagnosis of left atrial myxoma was not established for 8 months. This case history stresses the importance of considering the possibility of atrial myxoma in patients with heart murmurs whose symptoms, physical findings, and laboratory studies could be compatible with a systemic vasculitis.

Heart Atria↗
PubMed2001

Systemic vasculitis in patients with hepatitis C.

OBJECTIVE: To analyze the main characteristics of patients infected with hepatitis C virus (HCV) presenting with different types of vasculitis syndrome. METHODS: We retrospectively compared 2 groups of patients with HCV presenting with systemic vasculitis: 10 with biopsy proven polyarteritis nodosa-type systemic vasculitis (PAN, Group 1) and 7 with mixed cryoglobulinemia syndrome (MC, Group 2). RESULTS: Patients of Group 1 presented with different features than Group 2: life threatening systemic vasculitis (10 vs 0; p < 0.01), severe multifocal sensorimotor mononeuropathies versus distal moderate sensory polyneuropathies, malignant hypertension (5 vs 0; p = 0.04), cerebral angiitis (2 vs 0), ischemic abdominal pain (2 vs 0), kidney and liver microaneurisms (2 vs 0), increased erythrocyte sedimentation rate and C-reactive protein (7 vs 0; p < 0.01), renal insufficiency (5 vs 0; p = 0.04), HCV genotype 1b (3 vs 6; p = 0.06), and lower activity of chronic hepatitis (p = 0.02). Neuromuscular biopsies showed lesions of vasculitis in all patients, but the type of vasculitis was different in Group 1 compared to Group 2: medium size artery involvement (7 vs 0; p < 0.01), necrotizing vasculitis (10 vs 0; p < 0.01), and mononuclear cell infiltrate in perivascular areas (0 vs 7; p < 0.01). Using prednisone, plasma exchanges, and interferon-alpha, complete recovery was obtained in all PAN-type patients except one. In Group 2 patients, interferon-alpha did not have any effect on the peripheral neuropathy. CONCLUSION: HCV infection may be associated with different types of systemic vasculitis, i.e., polyarteritis nodosa or mixed cryoglobulinemia. Because of differences in clinical and pathological features and therapeutic strategy, PAN-type vasculitis should be distinguished from MC-type vasculitis in HCV patients.

Adult↗
PubMed2004

[Systemic vasculitis as an interdisciplinary problem].

Systemic vasculitis (SV) is characterized by generalized vascular bed lesion involving vessels of different sizes into a pathological process. The paper presents the results of a follow-up of 500 patients with different forms of SV, by making studies of immunity and the hemostatic system, angioscanning, Doppler ultrasound study of vessels, electrophysiological studies (rheoencephalography, encephalography), computed and magnetic resonance imaging of the brain, and visceral ultrasonography. A variety of clinical symptoms and involvement of different organs determine the interest of physicians of different specialties in the diagnosis and treatment of SV. The involvement of the nervous system in the process occurs in all forms of vasculitis, by afflicting the central, peripheral, and autonomic nervous systems with the development of regulatory and functional disorders. Lesions of the visual organ are typical of nonspecific aortoarteritis (Takayasu's disease), Wegener's granulomatosis, giant-cell arteritis. Recurrent uveitis is characterized in Behcet's syndrome. Cutaneous manifestations are included into the classification criteria of nodal polyartheritis, hemorrhagic vasculitis, and Kawasaki's disease. ENT and oral involvement are observed in Wegener's granulomatosis.

Arterial Occlusive Diseases↗
PubMed2002

Interferon-alpha and ribavirin treatment in patients with hepatitis C virus-related systemic vasculitis.

OBJECTIVE: Hepatitis C virus (HCV)-related vasculitis may involve multiple organs, including the skin, kidneys, and nervous system, and may be life-threatening. Although HCV is increasingly recognized as a cause of systemic vasculitis, limited data are available regarding the optimal treatment of this potentially serious condition. Therefore, we retrospectively analyzed the response to treatment in patients with chronic hepatitis C complicated by systemic vasculitis who had received antiviral therapy with interferon-alpha (IFNalpha) and ribavirin. METHODS: This retrospective study included 27 patients with systemic vasculitis and chronic HCV infection. Each patient had received treatment with IFNalpha and ribavirin for at least 6 months. The response to antiviral treatment was analyzed by comparing clinical, immunologic, and virologic data at the time of entry and during followup. Clinical response was defined according to the evolution of weight, arthralgia, nervous system, renal system, and cutaneous involvement. The virologic and immunologic responses were defined by the absence of HCV RNA and the absence of cryoglobulinemia, respectively, both 6 months after stopping antiviral therapy and at the end of followup. RESULTS: Patients received IFNalpha for a mean +/- SD of 20 +/- 14 months and ribavirin (at a mean +/- SD dosage of 895 +/- 250 mg/day) for 14 +/- 12 months. Other treatments included low-dose corticosteroids and plasma exchange. After a mean +/- SD followup of 57 +/- 29 months, 25 of 27 patients are alive and are being followed up as outpatients. Because of the heterogeneity of anti-HCV treatments received, the main results were stratified according to patients with 6 months of followup after stopping antiviral treatment (group 1, n = 14) and those who were still undergoing antiviral therapy at the time of analysis (group 2, n = 13). Nine patients in group 1 had a sustained virologic response and were clinical and immunologic complete responders. Four patients in group 1 were virologic nonresponders, and 3 of these patients had partial clinical and immunologic responses. Overall, 10 patients in group 1 had a complete clinical and immunologic response of their vasculitis (all 9 of the sustained virologic responders and 1 of the 5 patients who remained viremic). At the end of followup, 7 patients in group 2 were negative for HCV RNA; 6 were complete clinical responders. Among the other 6 patients in group 2, who had persistent viremia, 4 had a partial clinical response. Among the patients in group 1, HCV RNA was more often undetectable and genotype 1 was less frequent in complete clinical responders compared with partial/nonresponders. Age, sex, clinical vasculitic involvement, mean duration or total cumulative dose of IFNalpha or ribavirin, and use of steroids or plasmapheresis did not differ significantly according to clinical response. CONCLUSION: Treatment with IFNalpha and ribavirin can achieve a complete clinical response in most patients with HCV-related systemic vasculitis. Complete clinical response correlates with the eradication of HCV.

Adult↗
PubMed2005

Systemic vasculitis: still a challenging disease.

The efficacy of current therapeutic regimens in patients with systemic vasculitis is high, at the expense of considerable treatment toxicity. Optimal therapy for patients with these potentially life-threatening diseases is still debated. Our increased understanding of the pathogenetic mechanisms of systemic vasculitis has led to the development of new therapeutic alternatives, with better potential specificity for both the inflammation and immunologic causes of vasculitis: new immunosuppressive drugs (mycophenolate), monoclonal antibody modulators of lymphocyte function (rituximab), and cytokine-directed therapies (infliximab and eternacept). The safety and efficacy of such agents increasingly are being investigated in patients with systemic vasculitis. Additionally, many randomized prospective clinical trials have been completed to determine the precise roles for more conventional treatments (cyclophosphamide, azathioprine, and plasma exchange), providing an expanding evidence base to guide therapy in these challenging diseases. There now is clear evidence that duration of cyclophosphamide therapy can be relatively short (3 months), long-term maintenance therapy is needed to avoid relapse (often azathioprine), and patients at relatively greater risk for relapse can be identified. We review the most recent data on the current management of systemic antineutrophil cytoplasmic antibody-associated vasculitis, with emphasis on strategies to improve long-term outcome and reduce treatment toxicity while minimizing the risk for relapse.

Aged↗
PubMedDate not supplied

Remission in antineutrophil cytoplasmic antibody-associated systemic vasculitis.

The definition of remission in patients with systemic vasculitis must be distinguished from the term "cure," which implies that patients are well and not requiring ongoing therapy. Remission should be defined using a standardised approach to measuring clinical disease activity, and the definition should be qualified by the duration of the remission and the type of maintenance therapy required to sustain remission. Remission is an important goal of management in the systemic vasculitides and is achievable in most patients. Maintenance of remission is a more difficult target, and evidence from studies of patients with antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis indicates that durable, lasting remission is unlikely to occur. Despite good disease control, damage or scarring from disease or its treatment is a common finding and is a separate outcome from remission. Future studies of vasculitis therapies should address the concept of rapid and sustained disease control, so that patients spend most of their time in a state of good health, with minimal damage.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1994

[Sensitivity and specificity of antineutrophil cytoplasmic antibodies in systemic vasculitis].

The diagnostic value of c-ANCA as a specific marker of systemic vasculitis (particularly Wegener's granulomatosis) is well established. The prognostic value of c-ANCA for determining disease activity is controversial. We have prospectively studied in ten patients with systemic vasculitis over a mean period of 34 months (extreme 2-61 months). All patients had c-ANCA at the moment of the diagnosis: four patients had high titer of c-ANCA all over the period study; three clinical and biological exacerbations of the disease was observed without variation of the c-ANCA titer. In four patients c-ANCA disappeared within 6 months after the beginning of the treatment correlated with disease activity. Sometimes a rise of c-ANCA titer was observed with or without disease activity. In one case c-ANCA titer had a serrated evolution. The sensitivity and the specificity of the c-ANCA for disease activity in the ten studied patients were respectively 1 and 0.28. In patients with systemic vasculitis and c-ANCA at the time of the diagnosis, variation in c-ANCA titer alone is of limited prognostic value for predicting disease course.

Adolescent↗
PubMed1997

Necrotizing alveolar capillaritis in autopsy cases of microscopic polyangiitis. Incidence, histopathogenesis, and relationship with systemic vasculitis.

OBJECTIVES: To determine the incidence of necrotizing alveolar capillaritis, to elucidate its histopathogenesis and the most reliable histopathologic features for its detection, and to explore its relationship with systemic vasculitis in microscopic polyangiitis. METHODS: Twenty-five autopsy cases of microscopic polyangiitis were examined. Double staining with periodic acid-silver methenamine and trichrome was used. RESULTS: Periodic acid-silver methenamine and trichrome staining proved to be a useful and convenient method for the detection of necrotizing alveolar capillaritis. Capillaritis was detected in 10 (40%) of the 25 cases studied. Of 18 cases that exhibited the early degenerative stage of systemic vasculitis, capillaritis was seen in 10 (56%). Fibrinoid necrosis of the alveolar wall was the most reliable histopathologic feature for detecting necrotizing alveolar capillaritis in microscopic polyangiitis. The early stage of capillaritis displayed very little neutrophil exudation, and conspicuous accumulation of neutrophils developed after fibrinoid necrosis. Statistical analysis revealed that alveolar capillaritis had a statistically significant correlation (P < .05) with necrotizing vasculitis of the pulmonary circulatory system. Capillaritis tended to occur when systemic vasculitis of microscopic polyangiitis was in its early degenerative stage. CONCLUSION: Necrotizing alveolar capillaritis was detected in about half of the microscopic polyangiitis autopsy cases that exhibited the early degenerative stage of systemic vasculitis, and results based on earlier reports of clinical observations may have been too conservative.

Adult↗
PubMed2001

[Ophthalmologic manifestations of systemic vasculitis: report of six cases and review of the literature].

PURPOSE: Study of characteristics of ocular involvement in systemic vasculitis. METHODS: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. RESULTS: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. CONCLUSION: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.

Adrenal Cortex Hormones↗
PubMed1997

[Post-infectious systemic vasculitis: recovery without corticotherapy].

UNLABELLED: In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic. CONCLUSION: In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.

Adrenal Cortex Hormones↗
PubMed1994

Molecular analysis of C3 allotypes in patients with systemic vasculitis.

The third component of complement (C3) exists in two main allotypic forms, C3S and C3F, distinguished at the DNA level by a single base change. An increased frequency of the rarer C3F allele has been reported in patients with the autoantibody nephritic factor and in several other autoimmune conditions such as rheumatoid arthritis and IgA nephropathy. Studies of the immunogenetic factors predisposing to the development of systemic vasculitis have produced conflicting results and no major genetic predisposing factors have been identified. We have studied the C3S/F polymorphism in 63 patients with systemic vasculitis using DNA allotyping by the amplification refractory mutation system, a modification of the polymerase chain reaction. The allele frequency in these patients was C3S 0.71, C3F 0.29 (expected C3S 0.8, C3F 0.19; chi-squared = 5.1, P < 0.025), with the average relative risk for the development of systemic vasculitis associated with the presence of a C3F allele being 2.6. Moreover, there was a marked excess of C3FF homozygotes (11/63, [17.5%], versus 4% expected: chi-squared = 9.5, p < 0.01). The average relative risk for the development of systemic vasculitis in C3F homozygotes was 5.1, indicating a gene dosage effect. These data indicate that the C3F allele is associated with a predisposition to the development of systemic vasculitis and that C3F homozygotes are at particularly high risk. This association is the strongest genetic factor reported so far for this group of diseases.

Alleles↗
PubMed2000

Pathogenesis of ANCA-associated systemic vasculitis.

The aetiology of primary systemic vasculitides remains unknown. Recent advances have been made in the understanding of relevant mechanisms of inflammation, particularly the role of the endothelium and interactions with inflammatory mediators and immune effector cells. In Wegener's granulomatosis and microscopic polyangiitis the evidence suggests an autoimmune inflammatory process, characterized by an early lesion involving neutrophils and endothelial cells as both targets and active participants; priming of neutrophils and endothelial cells allows ANCA to activate neutrophils with damage localized to the endothelium. In the absence of immune complex deposition, the role of the ANCA is particularly intriguing. Endothelial cell damage and activation produces pro-inflammatory mediators with influx monocytes and T cells intensifying damage. Increased understanding of the pathogenesis of systemic vasculitis is likely to provide the basis for the use of more selective immunomodulatory therapies in the future.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1997

Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis.

Although cyclophosphamide and prednisolone are effective in treating systemic vasculitis, the optimum treatment regimes and duration of treatment are unknown. We randomized 54 patients aged 15-70 years (median 57.5 years) with systemic vasculitis (classical polyarteritis n = 8, microscopic polyarteritis n = 17, Wegener's granulomatosis n = 29) to treatment with either pulse cyclophosphamide and prednisolone (PCYP) (n = 24) or continuous oral and prednisolone and cyclophosphamide, with the latter followed after a median of 3 months (range 1.5-10 months) by azathioprine (CCAZP) (n = 30). Patients on CCAZP were more likely to develop leucopenia (13/30) than patients on PCYP, (7/24) although the difference was not significant. The numbers of infective episodes during follow up were comparable in the two groups at 1.7/patient for PCYP and 1.66/patient for CCAZP. Overall, 26/30 patients (87%) treated with CCAZP developed treatment-related toxicity, as did 17/24 patients (71%) treated with PCYP. After a median follow-up of 40.4 months (range 0.7-64.8), there was no difference in the frequency of deaths (PCYP 5, CCAZP 4), relapses (PCCYP 7, CCAZP 8), treatment failures (PCYP 4, CCAZP 4), improvement in disease activity scores or renal function. Survival at three years was 77% in patients treated with PCYP, and 90% in patients on CCAZP (p = 0.38). There was a tendency towards increased toxicity in patients treated with the continuous regimen.

Adolescent↗
PubMed1998

Evaluation, treatment, and prophylaxis of infections complicating systemic vasculitis.

Infection continues to be a major cause of morbidity and mortality in patients with systemic vasculitis. The recognition and treatment of infections in these patients is a particularly difficult task because the spectrum of potential pathogens is broad and the clinical manifestations of infection often mimic those of the underlying disease. This article provides 1) a general overview of the immunosuppressive properties of agents commonly used in the treatment of systemic vasculitis, and 2) a framework for the diagnostic evaluation and treatment of various infectious syndromes in patients with systemic vasculitis.

Evaluation Studies as Topic↗
PubMed1984

Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis.

Systemic vasculitis in rheumatoid arthritis shows similarities to polyarteritis nodosa and may require equally aggressive therapy. Forty-five patients with systemic rheumatoid vasculitis were studied during treatment with either cyclophosphamide plus methylprednisolone given by intermittent bolus intravenous injection (21 patients) or a variety of other more conventional drug regimens (24 patients). In this open study, the intravenous treatment group had more severe initial disease, a higher incidence of neuropathy, and more frequent evidence of necrotizing arteritis on biopsy than the other treatment group. Despite this, intravenous cyclophosphamide plus methylprednisolone resulted in more frequent healing of vasculitic lesions including leg ulcers and neuropathy, a lower incidence of relapse, fewer serious complications, and a lower mortality than did other treatments. Toxic effects were similar in both study groups. Intravenous cyclophosphamide plus methylprednisolone is a useful early treatment for systemic rheumatoid vasculitis.

Adult↗
PubMed2006

The role of biologic therapies in the management of systemic vasculitis.

The recent development of biologic therapies capable of selectively targeting components of the immune system has revolutionised the treatment of inflammatory arthritides. The steady increase in use of biologic agents coupled with the expansion in the knowledge of the pathogenesis of vascular inflammation has led to their application in the treatment of primary systemic vasculitis. These agents may have a role in addition to or in place of conventional immunosuppression and also be effective when the latter fails to induce remission. The use of biologics as targeted therapies has also, in reverse, improved our understanding of the pathophysiology of vascular inflammation. While the advent of biologics heralds a new era in the management of the systemic vasculitis, evidence for their efficacy is still in its infancy and has yet to match that of conventional immunosuppressants. In this review, we examine the up-to-date evidence for the use of biologics in systemic vasculitis, including TNF-alpha inhibitors, and highlight the challenges facing their use. We examine the rationale for using biologics based on the pathophysiology of vasculitis. Issues of toxicity and pharmacovigilance with the use of biologics are also discussed. Finally, future directions and predictions are presented.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1985

[Rheological changes in the blood and disseminated intravascular coagulation (DIC) in systemic vasculitis].

Eighty-six patients with different versions of systemic vasculitis were examined for rheological blood properties and some immunologic characteristics. The relationship between the formation of immune complexes and disorders of rheological characteristics and cell aggregation was established with emphasis on the necessity of correcting these disorders by anticoagulants and deaggregating agents for the treatment of patients with systemic vasculitis to be successful.

Adolescent↗