Different Types Of Vasculitis Research Articles

Diagnostic delays in systemic vasculitides.

Systemic vasculitides are among the less common disorders encountered in routine rheumatology practice. The low incidence and heterogeneous presentation at onset can potentially lead to delayed diagnosis. Not recognizing these in the early phase may prove detrimental, as some vasculitis may progress to a catastrophic course with major morbidity or mortality. The causes of diagnostic delay may vary among different types of vasculitis and may also be disease-, patient-, or physician-related. Disease-related factors include the myriad presentations with diverse and non-specific symptoms,mimicking other conditions like infections.In addition,some forms have prolonged prodromal phases before evident organ damage. Limited awareness among healthcare professionals,particularly outside rheumatology,and a lack of readily available diagnostic tools contribute to missed diagnoses. Delays in seeking care due to non-specific symptoms or lack of access to specialist care can worsen outcomes. The economic burden also increases with delayed diagnosis and damage accrual when the disease remains unrecognized or untreated for prolonged periods. Although the causes of vasculitis are numerous, including secondary causes, in this review, we focus on diagnostic delays in primary vasculitides and suggest potential steps to identify and treat these diseases early. These include educating both healthcare professionals and the public about the signs and symptoms of vasculitis; expanding the rheumatology workforce and facilitating timely referrals; implementing readily available and reliable tests for early detection; and streamlining care and diagnostic pathways. Such measures have the potential to improve the overall outcomes of the disease, with prolonged remission, minimal damage accrual, and improved quality of life.

Different Types of Vasculitis Complicated by Heparin-Induced Thrombocytopenia-Analysis of Four Cases and Literature Review.

Vasculitis and HIT have different etiologies, although both involve autoimmune mechanisms. Treatment of vasculitis often requires the use of an anticoagulant such as heparin, which can lead to the development of HIT and subsequent life-threatening complications. The analysis covered patients hospitalized in the Department of Internal Medicine, Nephrology and Dialysis in the period from September 2020 to March 2023. After analyzing the data, we selected four patients in whom vasculitis treatment was complicated by HIT. These included two patients with ANCA vasculitis and two patients with anti-GBM disease. We also described similar cases reported in the literature.

Ethnic association in primary systemic vasculitis: A systematic review

Background: Literature described wide disparities in incidence and prevalence between different types of vasculitis. There were no comprehensive studies on ethnic or racial associations in all types of primary systemic vasculitis (PSV) in any published article, until this review commenced in 2020. The purpose of the review is to synthesize the evidence regarding the relation of ethnicity and the incidence and/or prevalence of different types of PSV. Methods: A total of 52 selected articles which include Clinical trials, cohorts, cross-sectional studies, case series, and case studies and have been published within the last 10 years in the human population, were reviewed by searching The Medline, PubMed, and Google Scholars databases using predefined keywords. The PRISMA diagrams were followed to identify relevant articles. The methodological qualities of the studies were assessed using the EPHPP tool. Finally, a summary of the evidence on the association between ethnic origin and PSV was painstakingly compiled. Results: The connection between ethnicity and different types of PSV has been found to be significantly diverse in this research such as vasculitis is more common in Asians and Scandinavians, Kawasaki disease and periarteritis nodules are more prevalent in Japanese and Alaska-natives, ANCA-associated vasculitis is more frequent in Caucasians, whereas Henoch-Schonlein purpura and Cogan syndrome more usual in Caucasians and Asians. Furthermore, Behçet's disease more commonly occurs on the "Silk Road", especially in Turkey. Conclusion: Genetic susceptibility and environmental elements could be the contributing factors to the global variation in the incidence and prevalence of primary systemic vasculitis.

Ethnicity association in primary systemic vasculitis : A systematic search and review

Literature described wide disparities in incidence and prevalence between different types of vasculitis. There were no comprehensive studies on ethnic or racial associations in all types of primary systemic vasculitis (PSV) in any published article until this review commenced in 2020. The review aims to synthesize the evidence regarding the relation between ethnicity and the incidence and/or prevalence of different types of PSV. A total of 52 selected articles which include clinical trials, cohorts, cross-sectional studies, case series, and case studies and have been published within the last 10 years in the human population, were reviewed by searching Medline, PubMed, and Google Scholars databases using predefined keywords. The PRISMA diagrams were followed to identify relevant articles. The methodological qualities of the studies were assessed using the EPHPP tool. Finally, a summary of the evidence on the association between ethnic origin and PSV was painstakingly compiled. The connection between ethnicity and different types of PSV has been found to be significantly diverse in this researchas vasculitis is more common in Asians and Scandinavians, Kawasaki disease and periarteritis nodules are more prevalent in Japanese and Alaskanatives,ANCAassociated vasculitis is more frequent in Caucasians, whereas Henoch-Schonleinpurpura and Cogan syndrome more usual in Caucasians and Asians. Furthermore, Behçet's disease more commonly occurs on the "Silk Road," especially in Turkey. Genetic susceptibility and environmental elements could contribute to the global variation in the incidence and prevalence of primary systemic vasculitis.

The Impact of High Dose Glucocorticoids on Bone Health and Fracture Risk in Systemic Vasculitides.

Systemic vasculitides are a range of conditions characterized by inflammation of blood vessels which may manifest as single organ or life-threatening multisystem disease. The treatment of systemic vasculitis varies depending on the specific disease but historically has involved initial treatment with high dose glucocorticoids alone or in conjunction with other immunosuppressive agents. Prolonged glucocorticoid treatment is frequently required as maintenance treatment. Patients with small and large vessel vasculitis are at increased risk of fracture. Osteoporosis may occur due to intrinsic factors such as chronic inflammation, impaired renal function and to a large extent due to pharmacological therapy with high dose glucocorticoid or combination treatments. This review will outline the known mechanism of bone loss in vasculitis and will summarize factors attributing to fracture risk in different types of vasculitis. Osteoporosis treatment with specific consideration for patients with vasculitis will be discussed. The use of glucocorticoid sparing immunosuppressive agents in the treatment of systemic vasculitis is a significant area of ongoing research. Adjunctive treatments are used to reduce cumulative doses of glucocorticoids and therefore may significantly decrease the associated fracture risk in patients with vasculitis. Lastly, we will highlight the many unknowns in the relation between systemic vasculitis, its treatment and bone health and will outline key research priorities for this field.

Imaging features of 18F-FDG PET/CT in different types of systemic vasculitis.

To analyze 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) image features in different types of systemic vasculitis and explore its classification and diagnostic significance for systemic vasculitis. Clinical and 18F-FDG PET/CT imaging data of 71 consecutive patients (34 males, 37 females, mean age 53.4 ± 20.5years) who were examined at Peking University People's Hospital and diagnosed with active systemic vasculitis were analyzed retrospectively, and 18F-FDG PET/CT imaging features of different types of systemic vasculitis were analyzed in patients with positive 18F-FDG PET/CT findings. The proportions of large-, medium-, small-, and variable-vessel vasculitis were 36.6%, 15.5%, 29.6%, and 18.3%, respectively, and 93.0% of patients had positive findings on PET/CT, of which 63.6% had vascular involvement and 53.0% had extravascular involvement. Different types of vasculitis had different lesion sites, distribution patterns, and morphological changes. Large-vessel vasculitis mainly affected the aorta and its branches to the head and neck. Takayasu arteritis showed segmental involvement of the vascular and morphological changes of the vessel wall. Giant cell arteritis manifested as diffuse vascular involvement, which usually involved the temporal artery and the abdominal aorta and its branches. Medium-vessel vasculitis polyarteritis nodosa manifested as diffuse vascular involvement of both lower extremities. Small-vessel vasculitis anti-neutrophil cytoplasmic antibody-associated vasculitis manifested as granulomatous inflammation of extravascular regions. Variable-vessel vasculitis Behcet's disease involved both blood vessels and extravascular regions. Different types of systemic vasculitis show characteristic manifestations in 18F-FDG PET/CT images, which may be useful for the diagnosis and classification of systemic vasculitis. • Determining an early diagnosis of systemic vasculitis may be challenging. • Different types of systemic vasculitis show characteristic manifestations in 18F-FDG PET/CT images. • 18F-FDG PET/CT may be useful for the diagnosis and classification of systemic vasculitis.

Profile of pediatric vasculitides – Prospective hospital-based data from eastern India

Background: The pediatric vasculitides are multisystem autoimmune disorders involving the blood vessels of varying sizes. Wide geographic and ethnic variations are noted in terms of incidence and clinical features.Materials and Methods: This prospective, observational study was carried out over a period of 4 years from 2015 February to 2019 February in a tertiary care referral hospital to delineate the epidemiological pattern, clinical features, and pathological characteristics of pediatric vasculitides. All consecutive patients with vasculitis defined by the American College of Rheumatology and Chappel Hill Consensus Criteria diagnostic criteria were included in the study. They underwent relevant investigations and were treated as per the standard guidelines.Results: Of the total 110 patients with different types of vasculitis, 90% (n = 99) had primary vasculitis and the rest had secondary (10%, n = 11). Immunoglobulin A vasculitis (Henoch–Schonlein Purpura) (HSP) (n = 53, 48.19%) and Kawasaki disease (KD) (n = 35, 31.82%) were the most frequent types of the vasculitis followed by Takayasu arteritis and polyarteritis nodosa. Secondary vasculitides included those associated with systemic lupus erythematosus, juvenile idiopathic arthritis, scrub typhus infection, and drug penicillamine. Various typical and atypical features along with a definite seasonal pattern of their prevalence had been documented.Conclusion: A profile of pediatric vasculitides with a specific trend in their prevalence and seasonal pattern has been documented. HSP and KD constitute the predominant types of pediatric vasculitides. KD, although diagnosed more often than previous, HSP still outnumbers KD in Eastern India.

Systemic vasculitis and the gut

Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield. There are conflicting reports on the association of antineutrophilic cytoplasmic antibodies (ANCA) type with the frequency of gastrointestinal involvement in ANCA-associated vasculitis. Pancreatitis is a rare but serious complication of ANCA-associated vasculitis. Terminal ileitis may be observed in immunoglobulin A vasculitis and can be hard to distinguish from Crohn's disease. High fecal calprotectin levels can indicate active gastrointestinal involvement in both immunoglobulin A vasculitis and Behçet's syndrome. Refractory gastrointestinal involvement in Behçet's syndrome can be treated with thalidomide and/or TNF-α antagonists. The outcome of mesenteric vasculitis in systemic lupus erythematosus can be improved with high-dose glucocorticoids and cyclophosphamide or rituximab. Gastrointestinal system can be commonly involved in immunoglobulin A vasculitis, ANCA-associated vasculitis, polyarteritis nodosa, and Behçet's syndrome and can be an important cause of morbidity and mortality. Treatment depends on the type of vasculitis and is usually with high-dose corticosteroids and immunosuppressives.

Significance of isolated vasculitis in the gynecological tract: what clinicians do with the pathologic diagnosis of vasculitis?

Vasculitides includes a heterogeneous group of disorders with the common histologic findings of vascular wall inflammation. Systemic or localized disease (eg, renal vasculitis) has serious consequences. The incidence of isolated gynecologic vasculitis diagnosed on pathology specimens and its significance is little known. We performed a 20 year retrospective review including 53 cases with vasculitis diagnosis affecting the female genital tract identified in pathology reports. None had prior symptoms or were diagnosed with generalized vasculitis, while one patient had prior diagnosis of fibromyalgia. Most patients presented with abnormal bleeding and were treated for conditions unrelated to vasculitis. The different types of vasculitis were: predominantly lymphocytic (nonspecific) 30 cases, necrotizing 17 cases and granulomatous 6 cases. Only 2 patients had additional serologic tests. None of the patients with isolated gynecologic vasculitis received corticosteroids or additional treatment related to the vasculitis. None of the patients developed systemic vasculitis at follow-up (2 months-19.5 years; mean, 5.5 years). Isolated gynecologic vasculitis diagnosed on pathology slides is rarely associated with systemic vasculitis. Potential isolated gynecologic vasculitis causes include: previous surgical interventions and vascular inflammation secondary to local neoplasm. In almost all cases, clinicians did not perform a thorough laboratory analysis to exclude systemic vasculitis and therapy was not required in any case, suggesting minimal clinical significance.

Illness Perceptions and Fatigue in Systemic Vasculitis

To compare illness perceptions among patients with different forms of vasculitis, identify risk factors for negative illness perceptions, and determine the association between illness perceptions and fatigue. Participants were recruited from an online vasculitis registry to complete the revised Illness Perception Questionnaire (IPQ-R). The mean scores on each IPQ-R dimension were compared across different types of vasculitis. Cluster analysis and stepwise regression identified predictors of negative illness perception. Fatigue was measured using the general subscale of the Multidimensional Fatigue Inventory (MFI-20). Patient-reported measures of disease activity and IPQ-R dimensions were assessed in relation to MFI-20 scores using linear regression in sequential, additive models with model-fit comparisons. In total, 692 participants with 9 types of vasculitis completed the IPQ-R. For 6 of the 8 IPQ-R dimensions, there were no significant differences in mean scores between the different vasculitides. Scores in the identity and cyclical dimensions were significantly higher in Behçet’s disease compared with other types of vasculitis (13.5 versus 10.7 for identity and 4.0 versus 3.2 for cyclical [P < 0.05]). Younger age (odds ratio [OR] 1.04, 95% confidence interval [95% CI]1.02–1.06), depression (OR 4.94, 95% CI 2.90–8.41), active disease status (OR 2.05, 95% CI 1.27–3.29), and poor overall health (OR 3.92, 95% CI 0.88–17.56) were associated with negative illness perceptions. The sequential models demonstrated that the IPQ-R dimensions explained an equivalent proportion of variability in fatigue scores compared with measures of disease activity. Illness perceptions are similar across different types of vasculitis, and younger age is a risk factor for negative illness perceptions. Illness perceptions explain differences in fatigue scores beyond what can be explained by measures of disease activity.

Kawasaki disease and Henoch–Schönlein purpura – 10 years’ experience of childhood vasculitis at a university hospital in Taiwan

To investigate the clinical manifestations, disease activity and prognosis in different types of vasculitis. The charts of pediatric patients with vasculitis diagnosed from December 1997 to December 2007 were retrospectively reviewed. The first clinical manifestations and laboratory results were recorded at the time of diagnosis, and outcome evaluations with history of flare-ups were analyzed. A total of 508 vasculitis patients were included in this study, of whom 124 had Henoch-Schönlein purpura (HSP), and 351 had Kawasaki disease (KD). Hematuria was observed in 79% of recurrent HSP patients at the time of diagnosis, and was associated with an increased risk of relapse (p = 0.000). In Kawasaki disease, the clinical symptoms with erythematous changes in Bacille Calmette-Guérin scars and coronary artery dilatation were more prominent in patients younger than 1 year old, and lymphadenopathy was more common in patients older than 1 year old (p = 0.001). The risk of coronary dilatation was significant in the patients with an initial presentation of thrombocytosis, and greater in patients younger than 1 year old (p = 0.027). Thrombocytopenia was more prominent in vasculitis-associated autoimmune diseases. Marked lymphocytosis with increased C-reactive protein levels was significantly noted in urticarial vasculitis patients compared with HSP patients in multivariate logistic regression analysis. Vasculitis disease activity and prognosis were associated with initial laboratory results and clinical manifestations. Further large-scale clinical trials are warranted to validate these findings.

Nierenbeteiligung bei systemischen Vaskulitiden

The systemic vasculitides are a heterogenous group of autoimmune disorders, characterised by vessel wall inflammation leading to tissue damage and consecutive loss of function in the dependent organ systems. Vasculitides are classified by aetiological, morphological and immunological criteria. Primary and secundary vasculitis are distinguished diseases and they are categorised according to different sizes of the involved vessels into small, medium and large vessel vasculitis. Immunologically important are 2 different types of vasculitis, immune complex-mediated and pauci immune vasculitis. Renal involvement with rapid progressive glomerulonephritis (RPGN) is a life-threatening complication and needs to be diagnosed and treated immediately. Necrotising crescentic glomerulonephritis is histologically characteristic for ANCA-associated vasculitis. There are evidence-based protocols for induction and maintenance therapy. Combined treatment with steroids and cyclophosphamide is mandatory for critical organ involvement. B-cell-depleting therapy with rituximab is an efficient alternative. Azathioprine, methotrexate and leflunomide are recommended for maintaining remission. Infections, related to therapy or disease, are of particular clinical interest, because they are responsible for increased mortality during the early course of disease. Furthermore, gonadal insufficiency after cyclophosphamide therapy and steroid-induced osteoporosis should be prevented. Immune complex-mediated vasculitis could also cause renal involvement, although the course of glomerulonephritis is less agressive. Kryoglobulinaemic vasculitis is often associated with active hepatitis C infection and remission could be achieved by elimination of virus with interferon alpha. Renal involvement in large vessel vasculitis is mainly attributed to ischaemia and consecutive renal hypertension.

A Case of Microscopic Polyangiitis and Giant Cell Arteritis after Influenza Vaccination

We report a case of microscopic polyangiitis (mPA) and giant cell arteritis (GCA) (polyangiitis overlap syndrome) after influenza vaccination. A 67-year-old female with chronic kidney disease, who had been observed by a physician, presented fever and headache after immunization of influenza vaccine. She was diagnosed as having with mPA and GCA based on symptoms, worsening of renal function, biopsy of temporal artery (giant cell arteritis) and skin (microscopic polyangiitis), pulmonary involvement and the presence of myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). She was treated with prednisolone (PSL) and the symptoms were improved. However, two months later she was presented with general physical weariness. She was diagnosed as having with pneumocystis pneumonia, cytomegalovirus infection and cryptococcosis. Despite intensive treatment, she was died and autopsy was performed. The present case suggests that the influenza vaccination may cause different types of vasculitis, mPA and GCA, through the common mechanism in pathophysiology. This patient is also the first case of mPA and GCA proven by histological examination.

Infection and vasculitis

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.

Nodular vasculitis in systemic lupus erythematosus

A 42-year-old man presented with fever, photosensitivity, headaches, myalgia, hyperhidrosis, muscle weakness, alopecia, nasal crustae, weight loss, painful nails, arthritis, oral ulcers, erythema, discoid cutaneous lesions, and painful subcutaneous nodes. We made a diagnosis of systemic lupus erythematosus (SLE), type II cryoglobulinemia, and nodular vasculitis. In the skin, different types of vasculitis may be observed. Typically, histology shows leukocytoclastic vasculitis of superficial vessels both in SLE and mixed cryoglobulinemia, which clinically results in palpable purpura. In our patient, however, histopathological examination of the subcutaneous nodes not only revealed leukocytoclastic vasculitis of the superficial vasculature but also showed even more extensive involvement of dermal and subdermal small and medium sized vessels, giving rise to a nodular vasculitis.

Introduction: new trends in pregnancy and rheumatic diseases

Immunological and epidemiological evidence suggests that female sex hormones play an important role in the aetiology and pathophysiology of chronic inflammatory diseases; however, whether (or when) estrogens are friends or foes in inflammatory/ immune-mediated rheumatic diseases is still a matter of debate [1]. Several significant factors generate confusion and opposite conclusions might arise from the relatively superficial translation done from the animal studies to the human condition, the different effects of oestrogens on their different receptors or on different target cells, the different oestrogen concentrations employed and finally, opposite effects (especially on cell proliferation) exerted by different peripheral oestrogen metabolites. Pregnancy represents a singular condition of physiological interference on hormonal and immunological behaviour in females affected by chronic autoimmune diseases. For example, one paper in this issue discusses molecular and clinical disease activity markers in RA patients during the third trimester of pregnancy [2]. Furthermore, a further paper discusses when and whether autoantibodies might act as predictors of pregnancy complications [3]. Pregnancy in chronic autoimmune diseases such as SLE, SSc, RA and others is reviewed. Management of chronic autoimmune diseases and vasculitis in pregnancy is often a challenge. This is particularly true in the patient with organ involvement or organ damage. SLE shows a broad spectrum of disease manifestations ranging from mild symptoms to severe conditions that threaten maternal and fetal health. Similar risks are found in SSc and the different types of vasculitis. The APS alone or complicating the autoimmune diseases is characterized by thrombosis and pregnancy morbidity. Four papers deal with the problems associated with these conditions and address their management [4–7]. A multidisciplinary approach has been found essential in dealing with these highrisk pregnancies and achieving a successful outcome. Improved diagnostic skills in detecting impaired fetal health have helped to offer early treatment for children at risk though effective prevention of risk is still far away. After the introduction of biological agents, the management of several rheumatic diseases has changed dramatically. As a consequence of better control of disease activity more patients consider pregnancy. However, pregnancy experience with regard to new drugs is in general scanty and leaves both the patient with a desire for children and the treating physician in a dilemma. Two papers present the actual knowledge on the interaction of immunosuppressive drugs and reproduction [8, 9]. Importantly not only drug treatment during pregnancy and lactation, but also long-term effects on antenatally exposed offspring are discussed. Treatment of infertility in patients with autoimmune rheumatic

Refractory Central Nervous System Vasculitis and Gastrocnemius Myalgia Syndrome in Crohn's Disease Successfully Treated with Anti-Tumor Necrosis Factor-α Antibody

Secondary vasculitis represents a rare extraintestinal manifestation of Crohn's disease (CD). Appropriate and prompt diagnosis is often delayed by uncertainties about the relationship of the vasculitic manifestations and CD. To describe our experience with vasculitis in CD and review the literature with respect to different manifestations and pathophysiological aspects of extraintestinal vasculitic manifestations of CD. We report 2 new cases of CD with secondary small-vessel vasculitis. We also extensively review the literature (1960-2007) using a broad range of key words related to secondary vasculitis in CD. Relevant publications were evaluated for the number of reported patients and manifestations of vasculitis. Vasculitis is a rare extraintestinal manifestation of CD. Different types of vasculitis affect large-, medium-, and small-sized vessels associated with CD. Common immunologic features include intestinal inflammation as well as an infiltration of gammadelta-T-cells and/or Th1-type cells into vessel walls. The 2 new cases of secondary vasculitis in CD reported here reflect 2 major types of CD-related inflammatory vascular disorders. The first involves the central nervous system, while the second represents circumscribed Musculus gastrocnemius involvement (so-called "gastrocnemius myalgia syndrome"). Successful treatment of refractory secondary vasculitis in CD with an anti-tumor necrosis factor-alpha antibody is shown for the first time. Vasculitis secondary to CD is an uncommon finding. Therefore, it has to be carefully differentiated from other forms of primary or secondary vasculitis with intestinal involvement. Treatment with an anti- tumor necrosis factor-alpha antibody may prove a treatment option in vasculitis as an extraintestinal manifestation of CD.

Vasculitis in Systemic Lupus Erythematosus

We conducted the current study to determine the prevalence and clinical characteristics of vasculitis in a large series of patients with systemic lupus erythematosus (SLE), focusing on the classification and clinical significance of the different types of vasculitis. We studied 670 consecutive patients who fulfilled 4 or more of the 1997 revised criteria for SLE. Definite vasculitis was diagnosed histologically and/or by arteriography, and probable vasculitis was diagnosed clinically when there were characteristic cutaneous lesions. Vasculitides were categorized according to the definitions adopted by the Chapel Hill Consensus Conference. Seventy-six (11%) patients with SLE had vasculitis (68 female patients and 8 male; mean age, 37.8 yr); only 32 (42%) fulfilled the Chapel Hill definitions. Cutaneous lesions were the main clinical presentation of vasculitis, present in 68 (89%) patients, while the remaining 8 (11%) had isolated visceral vasculitis. Compared with SLE patients without vasculitis, patients with vasculitis had a higher prevalence of livedo reticularis (22% vs. 3%; p = 0.028); a higher mean European Consensus Lupus Activity Measurement (ECLAM) score (5.86 vs. 3.87; p < 0.001); and a higher frequency of anemia (62% vs. 17%; p < 0.001), erythrocyte sedimentation rate (ESR) >50 mm/h (60% vs. 15%; p < 0.001), and anti-La/SS-B antibodies (19% vs. 5%; p = 0.014) in the multivariate analysis. With respect to the size of the vessels involved, 65 (86%) patients had small vessel vasculitis (SVV) and 11 (14%) had medium-sized vessel vasculitis (MVV). SLE patients with MVV had a higher prevalence of mononeuritis multiplex (54% vs. 2%; p < 0.001), visceral vasculitis (100% vs. 5%; p < 0.001), and ulcerated/ischemic cutaneous lesions (36% vs. 11%; p = 0.047) and a higher percentage of surgical interventions (45% vs. 0%; p < 0.001) compared with patients with SVV. In conclusion, we observed a heterogeneous presentation of vasculitides arising in the setting of SLE, with nearly 60% of cases not fulfilling the names and definitions adopted by the Chapel Hill Consensus Conference. SVV was the most frequent vasculitis, overwhelmingly cutaneous and clearly differentiated from MVV, which was less frequent but had predominantly visceral involvement (especially of the peripheral nerves). The presence of vasculitis in our patients with SLE was associated with a higher ECLAM score, livedo reticularis, hematologic parameters (anemia, high ESR), and anti-La/SS-B antibodies.

The Primary Vasculitides

Primary vasculitis is the inflammation and necrosis of vessel walls not associated with infections, drugs, and autoimmune and lymphoproliferative disorders. It is important to make the correct diagnosis of different types of vasculitis, as their prognosis may be significantly different. Classification of vasculitis based on the size of the vessel is helpful, but there is often an overlap. Whereas the criteria proposed by the American College of Rheumatology are primarily clinical, the definitions set forth by the Chapel Hill Consensus Conference are based only on histologic observations. Correct diagnosis requires appropriate incorporation of the clinical history, laboratory parameters, and the histologic data. Incorporation of antineutrophil cytoplasmic antibodies in defining the pathogenesis of vasculitis has been particularly useful in diagnosing those small vessel vasculitides that are life threatening and need immediate intervention.

Plasma Exchange and Interferon-Alpha Pharmacokinetics in Patients with Hepatitis C Virus-Associated Systemic Vasculitis

Different types of vasculitis have been reported in association with hepatitis C virus (HCV) infection, i.e. type II mixed cryoglobulinemia and polyarteritis nodosa (PAN). Therapeutic approach of such severely symptomatic patients include interferon-alpha (IFN) plus plasma exchange (PE). There are no data on IFN pharmacokinetic changes related to PE. Patients and Methods: We studied 7 HCV-infected patients (mean age: 57 years) presenting with symptomatic type II mixed cryoglobulinemia (n = 5) or biopsy-proven PAN (n = 2). All patients underwent subcutaneous IFN therapy with concomitant PE. Serum IFN concentration was measured in serial samples on days with and without PE. Results: Without PE, IFN C_max(range: 100–750 IU/ml) was obtained 3–6 h after subcutaneous injection, followed by a 3- to 9-hour plateau. IFN concentration declined subsequently reaching a residual concentration 24 h after injection, ranging from 20 to 40 IU/ml. PE performed 6 h after IFN administration resulted in increased IFN clearance in that the concentration-time IFN-α area under the curve decreased from 3,005 IU·h/ml (1,563–4,614) on days without PE to 2,142 IU·h/ml (973–4,123) on day with PE. Conclusions: In patients with HCV-associated vasculitis, PE increase IFN clearance. Combined IFN-α and PE therapy schedule have to be further studied to optimize its biological activity.