Sekund?re Vaskulitiden und Vaskulitis ?Mimics“

Secondary vasculitis is a form of vasculitis for which an underlying disease is known. Diseases associated with secondary vasculitis include infections, drug hypersensitivity, malignancy, rheumatoid arthritis, collagen vascular disease and sarcoidosis. Moreover, there are numerous conditions that can mimic vasculitis clinically, in laboratory testing, radiographically and in histopathology. It is evident that distinguishing primary vasculitis from secondary vasculitis and also vascular inflammation from non-vasculitic disorders (vasculitis mimics) has significant therapeutic implications.     

肺血管炎的临床与影像学特点解析

肺部血管炎包括原发性与继发性两大类.继发性血管炎包括感染性疾病、结缔组织病、恶性肿瘤和过敏性疾病所致肺血管炎.原发性血管炎的分类通常根据受累血管的大小分为大血管炎、中血管炎和小血管炎.肺部血管受累常见于原发性大血管炎[大动脉炎(Takayasu arteritis),巨细胞动脉炎(giant cell arteritis,GCA),白塞病(Behcetdisease)]和原发性抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性小血管炎[肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA),显微镜下多血管炎(microscopic polyangiitis),嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)].原发性肺血管炎的影像学表现极具多样性,包括血管壁增厚、结节影、空洞、磨玻璃影和实变影等.原发性肺部小血管炎常导致弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH).相比于胸片,胸部CT更能够显示肺血管炎的病变特征和侵及范围.肺部血管炎的诊断极具挑战性,需要通过患者的临床特征、影像学特点、实验室检查结果和组织病理学特征作出综合判断.     

Systemic lupus erythematosus vasculitis: A current therapeutic overview

Opinion statement The development of systemic lupus erythematosus (SLE) vasculitis is of prognostic value. The earlier the vasculitis is treated, the better the prognosis for SLE. Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is rare. Skin SLE vasculitis is successfully treated with antimalarials, but its discontinuation may result in an SLE flare even among patients in remission. When visceral SLE vasculitis is encountered, or when a disease state is perceived to be life-threatening, a more aggressive therapy is warranted. A combination of medications, plasmapheresis, and intravenous immunoglobulin treatment, along with high-dose steroids and cytotoxic drugs, are typically employed in the treatment of severe SLE vasculitis. Finally, patients with SLE vasculitis may benefit from a number of autoimmune disease therapies currently under investigation, such as switching cytokine responses from Th1 to Th2, and the manipulation of toll-like receptors, chemokines, and FcR receptors. Specific B-cell therapies (eg, anti-Blys, B-cell depletion) may also emerge as potential treatments for SLE vasculitis.     

Les vasculites cutanées : aux frontières de l’allergie. Aspects nosologiques et physiopathologiques et prise en charge clinique

The classification of vasculitis is based more on histological criteria than on clinical criteria. Vasculitis is an inflammation of the vessel wall associated to a cell infiltration whose nature is variable according to space and time. Different types of vasculitides are then described: leucocytoclastic vasculitis, granulomatous and necrotizing vasculitis. Most of the vasculitides depend on immunological mechanisms, even though a triggering antigen is rarely found. Immune complex hypersensitivity and delayed-type hypersensitivity are the two main mechanisms involved in vasculitis, except for large vessel vasculitis. We distinguish three types of vasculitis: leukocytoclastic vasculitis (nodular purpura and associated diseases), granulomatous and necrotizing vasculitis (panarteritis nodosa, Wegener’s disease), and large vessel angeitis (Horton’s disease, Kawasaki’s disease and Buerger’s disease). Hypotheses on the pathophysiology of these vasculitides is discussed as well as the treatments.     

Vasculitis and inflammatory arthritis

Vasculitis has been described in most types of inflammatory arthritis. The best described and most widely recognised form is rheumatoid vasculitis. The incidence of systemic rheumatoid vasculitis has declined significantly following the general early use of methotrexate in the 1990s, and it is now a rare form of vasculitis. Treatment of rheumatoid vasculitis is conventionally with glucocorticoids and cyclophosphamide, but there is an increasing role for rituximab similar to that in other types of vasculitis. Despite these developments the mortality of rheumatoid vasculitis remains high. Vasculitis in other types of inflammatory arthritis is less well described and the treatment remains empirical.     

Glomerulonephritis in the vasculitides: advances in immunopathology

Systemic vasculitis refers to a condition of blood vessel inflammation, of which the causes are various. In a substantial number of cases, autoantibodies against neutrophil cytoplasm constituents (ANCAs) are present. The authors then refer to the systemic vasculitis as ANCA-associated systemic vasculitis. Renal disease is an unfavorable component, leading to dialysis dependency in a considerable number of patients. This review aims to summarize in brief what was reported about ANCA-associated vasculitis in the recent past. What the exact pathogenic role of ANCAs in the development of systemic vasculitis is remains uncertain, and it is still not clear how their presence leads to the histopathologic lesions called vasculitis.     

Vascularites double-positives ANCA et anti-MBG : mise au point sur les spécificités cliniques et thérapeutiques et comparaison aux deux vascularites éponymes

Double-positive vasculitis with anti-polynuclear cytoplasm (ANCA) and anti-glomerular basement membrane (GBM) antibodies is a rare entity of systemic vasculitis defined by the presence of ANCA and anti-GBM antibodies. The gradual accumulation of clinical and therapeutic data shows the usefulness of identifying and differentiating this entity from the two vasculitis respectively associated with the isolated presence of each of these two antibodies. Indeed, the double-positive ANCA and anti-GBM vasculitis appears to associate the characteristics of the demography and the extra-renal and pulmonary involvement of the ANCA-associated vasculitis on the one hand, and of the histological type and severe renal prognosis of the anti-MBG vasculitis on the other hand, with the renal involvement which is the only involvement consistently observed in double-positive vasculitis. The aim of this focus is to describe the epidemiological, clinico-biological, histological and prognostic characteristics of this entity, in light of recent literature and ongoing therapeutic changes in the two eponymous vasculitis.     

Vasculitis-induced colonic strictures

PURPOSE: The clinical presentations of gastrointestinal involvement from systemic vasculitis are diverse. Colonic involvement from systemic vasculitis is unusual. We report the first case of a symptomatic colonic stricture associated with rheumatoid vasculitis and another associated with systemic lupus erythematosus. METHODS: The clinical, radiologic, and histologic features of two cases of symptomatic colonic strictures secondary to colonic involvement with vasculitis are described. The literature covering gastrointestinal involvement from vasculitis in these conditions is reviewed. RESULTS: Surgical resection of the colonic strictures was required in both patients and had a satisfactory outcome. CONCLUSIONS: These cases provide further evidence of the protean clinical presentations of intestinal involvement in systemic vasculitis. Although immunosuppression has been shown to be of value in the treatment of vasculitis affecting the gastrointestinal tract, surgical resection is required for established strictures.     

Large artery vasculitis following recombinant hepatitis B vaccination: 2 cases

We describe 2 women who developed large artery vasculitis shortly after receiving recombinant hepatitis B vaccination. One patient developed Takayasu's arteritis, the other a vasculitis involving subclavian and renal arteries. Both developed renal failure. Whether the vasculitis was caused by the vaccination is not known. Although small vessel vasculitis following hepatitis B vaccination has been reported a number of times, large vessel vasculitis associated with hepatitis B vaccination has been reported only once. These cases suggest that large artery vasculitis should be added to the list of possible side effects of hepatitis B vaccination.     

Vaskulitis

A histopathologically confirmed biopsy is the gold standard for the diagnosis of vasculitis. Possible etiologies include primary systemic vasculitis, secondary vasculitis or isolated single organ vasculitis, although on histopathological grounds alone a clear differentiation is frequently not possible. The key criteria of morphological vasculitis work-up include vessel size, type of inflammation (granulomatous, necrotizing and/or leukocytoclastic) as well as the presence or absence of immune complexes and extravascular inflammatory changes. Together with the typical organ involvement and serological data, these criteria constitute the basis of vasculitis classification. Differential diagnostic overlaps and possible discrimination methods are presented. In the same way that the clinical approach of vasculitis patients is an interdisciplinary one, histopathology can only provide a definite diagnosis in combination with clinical and serological data. A conclusive morphological diagnosis depends on the right time of biopsy and the selection of appropriate biopsy material.     

Ocular vasculitis: a multidisciplinary approach

PURPOSE OF REVIEW: The ophthalmologist has direct visual access to inflamed vessels when examining the retina, and "vasculitis" in ophthalmology has so far mainly referred to retinal vasculitis. In the past few years the means to explore vasculitis in the ocular sphere have improved. Indocyanine green angiography now enables the analysis of choroidal inflammatory vasculopathy as well as vasculitis of the sclera (scleritis) and episclera (episcleritis) in addition to retinal vasculitis. Because vasculitis detected by the ophthalmologist can be the presenting sign of a systemic disease and has to be approached in a multidisciplinary fashion, the emerging term "ocular vasculitis," instead of retinal vasculitis, should be used in the future. The term covers vasculitis affecting all structures of the eye and the periocular tissues as detailed in this article. The ocular findings have to be integrated within the established and accepted classification of systemic vasculitis, which is divided into primary vasculitides, where the vessel itself is the target of the inflammatory reaction, and secondary vasculitides, caused by other inflammatory processes. This review will deal with recently published articles on ocular vasculitis, including its clinical aspects, its link with systemic diseases, and its investigation and management. The discussion will be conducted within the framework of the new classification put forward here. RECENT FINDINGS: Novel imaging techniques such as indocyanine green angiography have made it possible to explore inflammation of choroidal vessels and of scleral vasculitis in addition to retinal vasculitis, contributing to the global concept of ocular vasculitis. It has been shown, in particular, that the choriocapillaris, a vascular structure adjacent to the retina, can be the site of a primary inflammatory vasculopathy unrecognized so far. Most of the recent articles cited, however, deal not so much with new findings but with the integration of ocular pathologic changes into the systemic diseases they are part of. New knowledge about disease mechanisms and novel therapeutic modalities with biologic agents cited in this review are coming from other fields but have contributed to progress in the management of ocular vasculitis. SUMMARY: New investigational techniques of vasculitis in ocular structures other than the retina have contributed to the development of the global concept of ocular vasculitis. This review shows the importance of promoting a comprehensive and global classification of ocular vasculitis compatible with the concepts accepted for systemic vasculitis to contribute to its multidisciplinary approach.     

全身小血管炎合并严重心肌受累10例分析

目的 通过对小血管炎引起的严重心肌受累病例的分析提高对本病的认识.方法 总结北京协和医院近12年共10例小血管炎心肌受累病例临床资料及预后.结果 小血管炎心肌受累主要表现心肌收缩功能受损,心脏超声检查可早期诊断并监测病情,糖皮质激素治疗可改善心功能.结论 小血管炎心肌受累仍属罕见病,早期诊断、及时治疗可以改善症状和预后.     

Primary cutaneous small vessel vasculitis

Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Sch?nlein purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis is indicated for symptomatic or systemic involvement, because cutaneous small vessel vasculitis is frequently a self-limited, single episodic disease. Conservative, symptomatic treatment includes leg elevation, warming, antihistamines, and nonsteroidal anti-inflammatory drugs. For mild recurrent disease, colchicine, dapsone, and prednisone are first-choice agents. Systemic or severe cutaneous disease requires more potent immunosuppression (eg, prednisone, azathioprine, or mycophenolate mofetil). Plasmapheresis/plasma exchange and intravenous immunoglobulin are viable considerations for refractory disease, but are cumbersome and expensive modalities. There is insufficient evidence to advocate the use of new biological or monoclonal antibody therapies in primary cutaneous vasculitis.     

How to diagnose and treat secondary forms of vasculitis

Vasculitis is considered to be secondary when it arises either in the context of a pre-existing connective tissue disease, as a result of direct infection with a limited range of organisms, especially viruses, or when it arises in response to exposure to a number of medications. Rheumatoid vasculitis is probably the most widely recognised form of secondary vasculitis, and in this article we review the incidence, clinical features and management of this condition. Infections may either trigger or cause some types of vasculitis. Drug therapy is a common cause of limited forms of vasculitis and may enhance our understanding of the mechanism of these diseases. The premature development of atherosclerosis in patients with existing connective tissue diseases or indeed primary vasculitis has been recognised for some time, and the underlying mechanisms are currently being studied. An appreciation of the complex and varied pathophysiology of secondary vasculitis may further our understanding of primary vasculitis.     

Vasculitis associated with rheumatoid arthritis

Vasculitis may accompany rheumatoid arthritis. One must distinguish between vascular involvement associated with the pathogenesis of rheumatoid arthritis, isolated digital vasculitis, and the syndrome of clinical rheumatoid vasculitis. The cause of clinical rheumatoid vasculitis is unknown. High titers of rheumatoid factor, cryoglobulins, diminished circulating complement, an increased prevalence of HLA-DR4, and the pathologic findings suggest an immune etiology. However, similar, but perhaps less pronounced, abnormalities occur in uncomplicated rheumatoid arthritis, and these findings are not universal in complicating vasculitis. Classic cutaneous clinical manifestations include ischemic ulcers, digital gangrene, and palpable purpura. Mononeuritis multiplex is another classic presentation of rheumatoid vasculitis. Small digital infarctions may accompany other manifestations in clinical vasculitis or may occur alone as isolated digital arteritis, in which case the prognosis is relatively favorable. Weight loss, pleuritis, pericarditis, ocular inflammation, splenomegaly, hepatomegaly, and Felty's syndrome have also been reported in association with rheumatoid vasculitis. Although renal involvement has been considered unusual in rheumatoid vasculitis, several studies suggest that this may be more common than previously recognized. Ideally, a biopsy or an angiogram confirms the diagnosis of rheumatoid vasculitis, but often the diagnosis rests upon the clinical picture. In general, blind biopsies are not helpful, although one series indicated that a blind rectal biopsy may be an exception to this rule. An elevated erythrocyte sedimentation rate, increased C-reactive protein level, anemia, thrombocytosis, hypoalbuminemia, and a positive rheumatoid factor are common laboratory findings. Leukocytosis, hypergammaglobinemia, leukocytopenia, an elevated creatinine level, and minimal abnormalities of the urinary sediment also occur in patients with rheumatoid vasculitis. However, these abnormalities overlap in patients with uncomplicated rheumatoid arthritis, and their role in distinguishing rheumatoid vasculitis from uncomplicated rheumatoid arthritis is limited. Other immunologic tests have no established clinical role in diagnosing rheumatoid vasculitis. Therapy depends upon the clinical manifestation of rheumatoid vasculitis. Uncomplicated rheumatoid arthritis deserves appropriate therapy, and general attention to nutrition, cessation of tobacco, and control of blood pressure are indicated for all patients. Isolated digital vasculitis generally requires no more than the usual treatment for uncomplicated rheumatoid arthritis. Appropriate dermatologic management is indicated for ischemic ulcers. Most clinical experience in managing more symptomatic rheumatoid vasculitis has focused on glucocorticosteroids, D-penicillamine, and cytotoxic immunosuppressive drugs.(ABSTRACT TRUNCATED AT 400 WORDS)     

Localized vasculitis of the gastrointestinal tract

BACKGROUND AND OBJECTIVES: Isolated vasculitis of the gastrointestinal (GI) tract is a rare entity. Endoscopic biopsies have low sensitivity to diagnose intestinal vasculitis, even though the endoscopic findings may be suggestive of this condition. Our aims were to describe a case of biopsy-proven colonic leukocytoclastic vasculitis and review the literature. METHODS: A patient with biopsy-proven colonic leukocytoclastic vasculitis is described. A Medline database search of cases with localized GI vasculitis between January 1985 and September 2005 was conducted. RESULTS: A 32-year-old man was admitted to the hospital because of abdominal pain and diarrhea. A colonic biopsy showed leukocytoclastic vasculitis. There are very few articles on leukocytoclastic GI vasculitis as a separate disease, and most of them emphasize the difficulty in classification. Unlike our case, in former cases of localized vasculitis a diagnosis was made after surgery. Although our patient had steroid-refractory biopsy-proven isolated intestinal vasculitis, treatment with intravenous cyclophosphamide resulted in rapid resolution of symptoms and surgery was not required. CONCLUSIONS: In patients with abdominal pain a diagnosis of intestinal vasculitis should be considered. Immunosuppressive therapy allowed our patient to avoid surgery and may be similarly beneficial in other similar cases.     

Central nervous system vasculitis in children

Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.     

A proposal concept of a polygene network in systemic vasculitis: lessons from MRL mouse models.

In addition to the studies of cellular and molecular events in the pathogenesis of systemic vasculitis, a genome analysis of mouse models may shed some light on the complex clinicopathological manifestations of systemic vasculitis. In the study of susceptibility loci to vasculitis in MRL mouse models, we learned that systemic vasculitis developed in a cumulative effect of multiple gene loci, each of which by itself did not have a significant effect to induce the related phenotype, thus indicating a polygenic system. The mice developed vasculitis in an additive manner of multiple genes with a hierarchical effect. Some of the susceptibility loci seemed to be common to those in other collagen diseases as well. Moreover, the loci controlling tissue specificity of vasculitis were present. One of the positional candidate genes for vasculitis showed an allelic polymorphism in the coding region, thus possibly causing a qualitative difference in its function. As a result, a particular combination of polygenes with such an allelic polymorphism may thus play a critical role in leading the cascade reaction to develop vasculitis, and also a regular variation of systemic vasculitis. This is designated as the polygene network in systemic vasculitis.     

ANCA-associated vasculitis as paraneoplastic syndrome with colon cancer: a case report

Coexistence of a vasculitis and a neoplastic disease is rare and the pathogenesis is unknown. Most of these associations refer to leukocytoclastic or poliarteritis nodosa (PAN)-type vasculitis and hematological malignancies. There are few reports of vasculitis in patients with solid tumours and there are also few reports of paraneoplastic ANCA-associated vasculitis. We report a case of p-ANCA-positive vasculitis with peripheral nerve involvement associated with a colon cancer. Vasculitis resolved after corticoid treatment and surgical removal of the tumour.