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Vasculitis mimics

Eamonn S. Molloy and Carol A. Langford

Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Ohio, USA Correspondence to Carol A. Langford, Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, OH 44195, USA Tel: +1 216 445 6056; fax: +1 216 445 7569; e-mail: [email protected]

Current Opinion in Rheumatology 2008, 20:29­ 34

Purpose of review There are many disorders that may closely resemble the clinical, radiologic and/or pathologic features of the primary vasculitides. In this review, we focus on recently described and under-recognized syndromes that may mimic vasculitis. Recent findings Hereditary causes of large-artery aneurysms such as Marfan's syndrome have long been recognized; recent years have seen a greater understanding of the genetics of Marfan's and other such disorders, including Loeys­Dietz syndrome and Ehler­Danlos syndrome type IV. Under-recognized mimics of medium-vessel vasculitis include segmental arterial mediolysis and Grange syndrome. A large number of entities can mimic small-vessel vasculitis. Recent descriptions of antibodies to human neutrophil elastase have provided insight into the occurrence of antineutrophil cytoplasmic antibodies in cocaine-induced midline destructive lesions. The differential diagnosis of cerebral vasculitis can be particularly difficult. Reversible cerebral vasoconstriction syndromes represent an important class of entities that can readily mimic cerebral vasculitis but have a very different management approach and outcome. Summary The diagnosis of vasculitis requires careful assessment of all available clinical, laboratory, radiologic and pathologic information, and consideration of many competing differential diagnoses. Awareness of noninflammatory mimics of vasculitis is essential to avoid unnecessary and potentially harmful treatment with immunosuppressive agents. Keywords aortic aneurysm, cerebral vasculitis, mimics, vasculitis

Curr Opin Rheumatol 20:29­34 ß 2008 Wolters Kluwer Health | Lippincott Williams & Wilkins 1040-8711

Introduction

Vasculitis is characterized by blood-vessel inflammation, which may lead to tissue injury from vascular stenosis, aneurysm, or rupture. Similar vascular consequences may also result from other disease processes that are not necessarily associated with blood-vessel inflammation. Many of these diseases may have clinical, laboratory, radiographic and/or pathologic findings in common with vasculitis, and therefore may lead to diagnostic confusion. Consequently, vasculitis can only be reliably diagnosed after careful consideration of all relevant diagnostic information. Even when a diagnosis of vasculitis is reached, secondary causes of vasculitis including drug reactions, infections and malignancy must be considered. Treatment of vasculitis mimics with immunosuppressive drugs may be ineffective or may worsen the disease, emphasizing the importance of an accurate diagnosis. In this review, we categorize vasculitic mimics based upon the predominant size of vessel involvement, with an additional focus on central nervous system (CNS) vasculitis. Detailed discussion is limited to selected entities that have been the subject of recent important publications.

1040-8711 ß 2008 Wolters Kluwer Health | Lippincott Williams & Wilkins

Large-vessel vasculitis

Disorders that are characterized by large-vessel vasculitis include giant-cell arteritis and Takayasu's arteritis. In patients who present solely with large-vessel involvement, tissue biopsies are not possible and the diagnosis is made on the basis of characteristic arteriographic changes combined with consistent clinical features. There are a number of other important disease processes that must be considered in the differential diagnosis of large-vessel vasculitis (see Table 1). Genetic disorders that can affect the large-sized and medium-sized vessels can mimic primary vasculitic diseases and include several entities for which recent insights have been made.

Genetic diseases that can affect the large vessels

Marfan's syndrome is a well recognized cause of ascending thoracic aneurysms and dissection (TAAD) and is attributable to mutations in the fibrillin 1 (FBN1) gene [1,2]; FBN1 mutations have also been described in patients with TAAD in the absence of the typical marfanoid phenotype [3,4]. Ehler­Danlos syndrome (EDS) type IV is an autosomal dominant disorder that results from mutations in the

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30 Vasculitis syndromes Table 1 Mimics of large-vessel vasculitis Infectious causes Acute (e.g. mycotic aneurysms associated with septicemia or endocarditis) Chronic (e.g. syphilis, tuberculosis, HIV, and leprosy) Atherosclerosis Congenital causes Aortic coarctation Middle aortic syndrome Hereditary disorders Marfan's syndrome Neurofibromatosis Ehler­Danlos syndrome (types IV and VI) Loeys­Dietz syndrome Pseudoxanthoma elasticum Fibromuscular dysplasia Iatrogenic Postradiation therapy Chronic periaortitis/inflammatory aortic aneurysma

a Some of these disorders may be associated with histologic findings suggestive of vasculitis. a

Table 2 Mimics of medium-vessel vasculitis Viral-associated vasculitisa HBV HCV HIV Herpes viruses Other infectious processesa Infective endocarditis Mycotic aneurysms Atherosclerosis Malignancya Lymphoma Leukemia Fibromuscular dysplasia (includes segmental arterial mediolysis) Hereditary disorders Ehler­Danlos syndrome Neurofibromatosis Grange syndrome Iatrogenic (postprocedural) Hypercoagulable states Thrombotic thrombocytopenic purpura Antiphospholipid syndrome

a

type III procollagen gene (COL3A1). The diagnosis of EDS IV may be confirmed by demonstration of production of abnormal type-III procollagen from cultured fibroblasts and by identification of the COL3A1 mutation. Unlike other types of EDS, EDS IV is not typically associated with joint hypermobility but can be associated with skin and facial changes. EDS IV is often diagnosed in adulthood or post-mortem, with death occurring from visceral or arterial rupture. Arterial complications may include aneurysm, dissection and caroticocavernous sinus fistulae; however, arteries may rupture even when there is little or no aneurysmal change [5]. Patients with EDS IV can also present acutely with life-threatening rupture of the intestines, the gravid uterus or other viscera [5]. Loeys­ Dietz syndrome was described in 2005 [6] and is associated with aortic and other arterial aneurysms, generalized arterial tortuosity, bifid uvula, cleft palate, craniosynostosis, congenital heart disease and learning disability. Loeys­Dietz syndrome is an autosomal dominant disorder associated with mutations in either of the transforming growth factor b receptors (TGFBR1/TGFBR2). TGFBR1/2 mutations have also been described in patients with the clinical phenotype of Marfan's syndrome [7] and EDS IV [8] without evidence of FBN1 or COL3A1 mutations respectively, emphasizing the overlap between these disorders. Risk for arterial and other complications and surgical outcomes differ depending on the specific genotype, strengthening the rationale for the performance of genetic testing in such cases to ensure accurate diagnosis.

Some of these disorders may be associated with histologic findings suggestive of vasculitis.

is often not feasible or is uninformative, however, requiring a dependence on angiographic findings to support the diagnosis. In such cases, consideration of competing diagnoses (Table 2) and assessment of the clinical response to immunosuppressive therapy, if instituted, are essential. Two under-recognized mimics of medium-vessel vasculitis are now discussed.

Segmental arterial mediolysis

Medium-vessel vasculitis

The prototypic medium-vessel vasculitis in adults is polyarteritis nodosa (PAN). In cases of PAN where there is skin, muscle or peripheral-nerve involvement, tissue biopsy can confirm the diagnosis in a patient with a compatible clinical picture. Biopsy of accessible tissues

Segmental arterial mediolysis (SAM; previously referred to as segmental mediolytic arteritis or segmental mediolytic arteriopathy) was initially described by Slavin et al. in 1976 [9] and may mimic PAN [10]. SAM is a noninflammatory, histologically defined vasculopathy that affects the medium-sized vessels supplying the gastrointestinal tract. The etiology of SAM is unknown, but is thought by some to be a variant of fibromuscular dysplasia [11,12], or possibly a consequence of vasospasm [13]. There is no demonstrated association with systemic infection, autoimmune diseases or hypertension, and neither has a genetic basis been defined to date. Most patients with SAM are in mid-to-late adulthood with equal male/female distribution. Patients typically present with intra-abdominal or retroperitoneal hemorrhage due to rupture of a visceral artery aneurysm. The cerebral vasculature is the second most commonly affected vascular bed, although involvement together with the abdominal vessels is rare. More recently, the occurrence of a venous angiopathy in SAM has been reported [13]. The abdominal angiographic features in SAM are indistinguishable from those of PAN, with multiple areas of ectasia and aneurysms. The diagnosis of SAM rests on the pathologic findings, which include noninflammatory lysis of the arterial media, separation at the medial-adventitial junction, and a florid reparative response In some cases, organizing thrombus and reactive changes at the site of an aneurysm rupture

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Vasculitis mimics Molloy and Langford 31

may be misconstrued as evidence of vasculitis, but in contrast to vasculitis, the vessel wall in SAM lacks transmural inflammation and fibrinoid necrosis. Although there are currently only approximately 45 cases of SAM reported in the English-language literature, it may be under-diagnosed in patients who do not require abdominal surgery as it is purely a histological diagnosis. Therefore, SAM must be considered in the differential diagnosis of cases of PAN that are limited to the gastrointestinal tract without clear-cut involvement of other organ systems. As SAM is a noninflammatory vasculopathy, there is no established role for immunosuppressive therapy. Although the natural history of SAM is poorly understood, no cases of recurrent hemorrhage have yet been reported.

Grange syndrome

Grange syndrome is a recently described hereditary disorder that is associated with a variable combination of multiple arterial stenoses and aneurysms, brachysyndactyly, bone fragility, learning disability and cardiac defects [14­16]. The arterial disease commonly results in a syndrome that resembles fibromuscular dysplasia, with hypertension and angiographic evidence of `beading'. No underlying genetic defect has yet been determined in Grange syndrome, but it is thought to be inherited in an autosomal recessive fashion.

The distinction between Wegener's granulomatosis and CIMDL is made more difficult by the finding of ANCA positivity in a significant proportion of patients with CIMDL [18­20]. In a recent study that compared 25 patients with CIMDL with a control cohort including 64 patients with Wegener's granulomatosis and 14 with MPA [20], Wiesner et al. demonstrated that 84% of patients with CIMDL tested positive for ANCA, with specificity for human neutrophil elastase (HNE). Perinuclear staining was the most common pattern seen on indirect immunofluorescence of ethanol-fixed neutrophils, although the pattern varied between perinuclear and cytoplasmic staining, even on serial samples from individual patients. None of the 95 serum samples obtained from the 25 patients with CIMDL had positive myeloperoxidase by ELISA. Of the 21 HNE ANCA-positive samples, 12 (57%) also reacted with proteinase 3 (PR3). Of note, none of the patients with Wegener's granulomatosis or MPA had positive results for HNE ANCA. Furthermore, only three of 118 patients with other forms of vasculitis and autoimmune diseases tested positive for HNE ANCA; two of these patients had ulcerative colitis, and one had ulcerative esophagitis. These findings suggest that testing for HNE specificity, but not PR3 specificity, may be useful in differentiating CIMDL from Wegener's granulomatosis.

Cerebral vasculitis Small-vessel vasculitis

The primary systemic small-vessel vasculitides are Wegener's granulomatosis, microscopic polyangiitis (MPA) and Churg­Strauss syndrome; other forms of vasculitis that may target small vessels include Behcet's ¸ ¨ syndrome, Henoch­Schonlein purpura, cryoglobulinemic vasculitis and cutaneous leukocytoclastic angiitis. The differential diagnosis for an individual patient with suspected small-vessel vasculitis will vary greatly depending on the pattern of organ and tissue involvement, the specific nature of the tissue injury, and the results of relevant laboratory, radiologic and pathologic studies. Testing for antineutrophilic cytoplasmic antibodies (ANCA) can provide valuable diagnostic information for patients with suspected systemic small-vessel vasculitis. Here we discuss a recently described pitfall of ANCA testing.

Cocaine-induced midline destructive lesions

It has long been recognized that nasal cocaine abuse can lead to destruction of the nasal septum, the palate and other sinonasal bony structures [17], and biopsy of sinonasal tissue may reveal necrotizing inflammation. Although Wegener's granulomatosis does not lead to perforation of bony structures other than the lamina papyracea, cocaine-induced midline destructive lesions (CIMDL), lymphoproliferative diseases, and infections enter the differential diagnosis of Wegener's granulomatosis in patients with localized sinonasal involvement.

Vasculitis affecting the CNS may arise as a component of primary systemic vasculitic diseases or rarely as a primary angiitis of the CNS (PACNS) where involvement is limited to the CNS vessels. Secondary CNS vasculitis may also occur in a wide range of connective-tissue diseases, as well as in the setting of infection or malignancy. The differential diagnosis of CNS vasculitis requires careful clinical assessment, neuroimaging, cerebrospinal-fluid analysis and, in selected cases, cerebral angiography or brain biopsy. The limitations of noninvasive testing for the diagnosis of CNS vasculitis have previously been highlighted [21]. A cerebral angiogram with `high probability' of vasculitis is more commonly found in nonvasculitic disorders due to vasospasm or atherosclerosis, and the `gold standard' of a brain biopsy may have false negative results because of sampling error. As a result of the suboptimal performance of the available diagnostic modalities, many diverse disorders must be considered in the differential diagnosis of CNS vasculitis (Table 3). Here we discuss a number of recently described syndromes that can mimic CNS vasculitis, either as a separate entity or in the setting of a preexisting vasculitic or connective-tissue disease.

Reversible cerebral vasoconstriction syndromes

In the early 1990s, a less severe subset of PACNS was recognized and termed benign angiopathy of the central nervous system (BACNS) [22]. Similar clinical

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32 Vasculitis syndromes

Table 3 Mimics of central nervous system vasculitis Other multisystem inflammatory disordersa Sarcoidosis Susac's syndrome Infectiona Bacterial Mycobacterial Fungal Viral Protozoal Malignancya CNS lymphoma Glioma Angiocentric lymphoma Vasospastic disordersa Reversible cerebral vasoconstrictive syndrome Drug exposures Other arterial disease Atherosclerosis Fibromuscular dysplasia Dissection Hypercoagulable states Thrombotic thrombocytopenic purpura Antiphospholipid antibody syndrome Stroke-like syndromes CADASIL Mitochondrial diseases Sickle cell disease Fabry's disease Sneddon's syndrome Leukoencephalopathies Progressive multifocal leukoencephalopathy Reversible posterior leukoencephalopathy syndrome Cerebral hemorrhagea Hypertensive Aneurysmal Amyloid angiopathy Arteriovenous malformation Moya-moya disease Embolic diseasea Thrombus Cholesterol Myxoma Endocarditis

a

though as RCVS is primarily treated with calcium channel blockers, typically verapamil, and cytotoxic therapy is not indicated.

Cerebral amyloid angiopathy

Cerebral amyloid angiopathy (CAA) is characterized by deposition of congophilic material in small-sized to medium-sized cerebral blood vessels. CAA is typically asymptomatic, but may cause lobar intracerebral hemorrhage and can be associated with intracranial mass lesions and Alzheimer's disease. Sporadic and familial forms of CAA have been reported. CAA may be associated with a variable degree of inflammatory cell infiltration ranging from none to mild perivascular infiltrates through to frank granulomatous angiitis [25,26,27,28]. Therefore, for patients with suspected CNS vasculitis, particularly those over the age of 40, brain-biopsy specimens should routinely be stained for amyloid deposition.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Some of these disorders may be associated with histologic findings suggestive of vasculitis.

and radiographic findings were also described in patients with a number of other syndromes including Call­ Fleming syndrome, postpartum angiopathy, migranous vasospasm and drug-induced `arteritis'. The characteristics of these entities were recently reviewed and collectively grouped under the term reversible cerebral vasoconstriction syndromes (RCVS) [23]. Patients with RCVS typically have abrupt onset of severe headache, closely followed, in 40% of cases, by cerebral infarction or occasionally, hemorrhage [24]. Spinal-fluid examination is normal and brain biopsy, if performed, is negative. Arteriography typically reveals alternating areas of ectasia and narrowing in multiple vascular beds. To satisfy the informal diagnostic criteria for RCVS, these arteriographic findings must be reversible within 3­4 months of symptom onset. RCVS is often mistaken for CNS vasculitis because of its clinical features and arteriographic findings. This distinction is of critical importance

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare disorder initially described in the 1970s that was recently found to be associated with mutations in the notch 3 gene on chromosome 19 [29]. CADASIL is a generalized small-vessel arteriopathy, but clinical manifestations are largely confined to the CNS. Common manifestations include migraine, ischemic strokes, transient ischemic attacks, cognitive impairment, psychiatric disturbance, seizures, coma, and cerebral hemorrhages. The diagnosis of CADASIL may be suspected on the basis of these clinical features with a positive family history of stroke. Typical MR findings of CADASIL are of lacunar infarcts and T2 hyperintensities in the subcortical white matter, and to a lesser extent the subcortical gray matter and brain stem; findings of bilateral anterior temporal white matter and external capsule involvement are more specific for CADASIL. Confirmation by genetic testing is possible in the majority of patients; the finding of granular osmophilic material within the vascular basal lamina on electron microscopy of skin-biopsy specimens can confirm the diagnosis in the remainder [30]. Disease severity and length of survival are highly variable [31]. There is no inflammatory component and thus no role for immunosuppressive therapy in the treatment of CADASIL.

Susac's syndrome

Susac's syndrome is a microangiopathy of unclear etiology that is more frequently reported in young adult females. Susac's syndrome is characterized by the typical clinical triad of acute or subacute encephalopathy, branch retinal artery occlusions and sensorineural hearing loss that results from small infarcts in the brain, retina and cochlea [32,33].

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Vasculitis mimics Molloy and Langford 33

Other acronyms by which this disease is known include SICRET (small infarctions of cochlear, retinal and encephalic tissue) syndrome or REDM (retinopathy, encephalopathy, deafness and associated microangiopathy) syndrome. Typical findings at MRI are of multiple small white-matter hyperintensities; gray-matter involvement may also be seen. Linear lesions in the corpus callosum are common and appear to be relatively specific for this disease. Cerebrospinal-fluid analysis usually reveals a lymphocytic pleocytosis and elevated protein levels. Histologic evidence of microangiopathic infarcts is seen, without evidence of vasculitis or thrombosis. While there are reports of efficacy of glucocorticoids and other immunosuppressive therapies in patients with Susac's syndrome [34], the assessment of these therapies is confounded by the fluctuation in disease course that may occur even without therapy.

Progressive multifocal leukoencephalopathy

Conclusion

There are many disorders that may mimic the clinical, laboratory, radiologic and/or pathologic features of the primary vasculitides. Awareness of these mimics is essential to avoid the use of unnecessary and potentially harmful immunosuppressive drugs and to direct management to the correct underlying cause of disease.

References and recommended reading

Papers of particular interest, published within the annual period of review, have been highlighted as: of special interest of outstanding interest Additional references related to this topic can also be found in the Current World Literature section in this issue (p. 114). 1 Dietz HC, Cutting GR, Pyeritz RE, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature 1991; 352:337­339. Lee B, Godfrey M, Vitale E, et al. Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes. Nature 1991; 352:330­334. Francke U, Berg MA, Tynan K, et al. A Gly1127Ser mutation in an EGF-like domain of the fibrillin-1 gene is a risk factor for ascending aortic aneurysm and dissection. Am J Hum Genet 1995; 56:1287­1296. Milewicz DM, Michael K, Fisher N, et al. Fibrillin-1 (FBN1) mutations in patients with thoracic aortic aneurysms. Circulation 1996; 94:2708­2711. Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000; 342:673­680. Loeys BL, Chen J, Neptune ER, et al. A syndrome of altered cardiovascular, craniofacial neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet 2005; 37:275­281. Mizuguchi T, Collod-Beroud G, Akiyama T, et al. Heterozygous TGFBR2 mutations in Marfan syndrome. Nat Genet 2004; 36:855­860.

2

Progressive multifocal leukoencephalopathy (PML) is a rapidly progressive, generally fatal, neurologic disease due to reactivation of the JC polyoma virus. PML may occur in patients with connective-tissue diseases, in particular, systemic lupus erythematosus (SLE), even on a background of minimal immunosuppressive therapy, suggesting that immune dysregulation may predispose to PML [35]. PML must be considered in patients with an underlying vasculitic or connective-tissue disease presenting with progressive neurologic deficits, even when background immunosuppressive therapy is modest. Suspicion of PML is heightened if progressive neurologic decline occurs following institution of aggressive immunosuppressive therapy. The diagnosis of PML can generally be confirmed by testing of cerebrospinal fluid for JC virus by polymerase chain reaction; however, if PCR testing is negative and clinical suspicion of PML remains high, brain biopsy should be performed. If a diagnosis of PML is confirmed, immunosuppressive therapy should be withdrawn.

Reversible posterior leukoencephalopathy syndrome

3

4 5

6

7 8

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10 Chan RJ, Goodman TA, Aretz TH, Lie JT. Segmental mediolytic arteriopathy of the splenic and hepatic arteries mimicking systemic necrotizing vasculitis. Arthritis Rheum 1998; 41:935­938. 11 Lie JT. Segmental mediolytic arteritis. Not an arteritis but a variant of arterial fibromuscular dysplasia. Arch Pathol Lab Med 1992; 116:238­241. 12 Slavin RE, Saeki K, Bhagavan B, Maas AE. Segmental arterial mediolysis: a precursor to fibromuscular dysplasia? Mod Pathol 1995; 8:287­294. 13 Slavin RE, Inada K. Segmental arterial mediolysis with accompanying venous angiopathy: a clinical pathologic review, report of 3 new cases, and comments on the role of endothelin-1 in its pathogenesis. Int J Surg Pathol 2007; 15:121­134. An update on the clinical and pathologic features of segmental arterial mediolysis. 14 Grange DK, Balfour IC, Chen SC, Wood EG. Familial syndrome of progressive arterial occlusive disease consistent with fibromuscular dysplasia, hypertension, congenital cardiac defects, bone fragility, brachysyndactyly, and learning disabilities. Am J Med Genet 1998; 75:469­480. 15 Wallerstein R, Augustyn AM, Wallerstein D, et al. A new case of Grange syndrome without cardiac findings. Am J Med Genet A 2006; 140:1316­ 1320. 16 Weymann S, Yonekawa Y, Khan N, et al. Severe arterial occlusive disorder and brachysyndactyly in a boy: a further case of Grange syndrome? Am J Med Genet 2001; 99:190­195. 17 Vilensky W. Illicit and licit drugs causing perforation of the nasal septum. J Forensic Sci 1982; 27:958­962. 18 Simsek S, de Vries XH, Jol JA, et al. Sino-nasal bony and cartilaginous destruction associated with cocaine abuse, S. aureus and antineutrophil cytoplasmic antibodies. Neth J Med 2006; 64:248­251.

Reversible posterior leukoencephalopathy syndrome (RPLS), initially described in 1996, clinically presents with headache, seizures, altered mental status, and visual and motor deficits [36]. Typical MRI findings in reversible posterior leukoencephalopathy syndrome are of reversible white-matter edema affecting the parietal and occipital lobes. A recent review has highlighted the occurrence of RPLS in 13 patients with systemic autoimmune conditions such as SLE and Wegener's granulomatosis [37]. Cofactors including accelerated hypertension, renal failure and immunosuppressive medications such as cyclophosphamide and cyclosporine were present in all cases. All but one of these patients improved with supportive measures, including hemodialysis, blood-pressure control and withdrawal of the offending agent.

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34 Vasculitis syndromes

19 Trimarchi M, Gregorini G, Facchetti F, et al. Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine (Baltimore) 2001; 80:391­404. 20 Wiesner O, Russell KA, Lee AS, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaineinduced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 2004; 50:2954­2965. 21 Duna GF, Calabrese LH. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol 1995; 22:662­667. 22 Calabrese LH, Gragg LA, Furlan AJ. Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system. J Rheumatol 1993; 20:2046­2050. 23 Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007; 146:34­44. This paper defines the eversible cerebral vasoconstriction syndrome, an important mimic of CNS vasculitis. 24 Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and longterm followup. Arthritis Rheum 2002; 47:662­669. 25 Eng JA, Frosch MP, Choi K, et al. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004; 55:250­256. 26 Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angio pathy-related inflammation. Neurology 2007; 68:1411­1416. This paper describes the salient features of cerebral amyloid angiopathy-related inflammation. 27 Schwab P, Lidov HG, Schwartz RB, Anderson RJ. Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum 2003; 49:421­427. 28 Scolding NJ, Joseph F, Kirby PA, et al. A beta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005; 128 (Pt 3):500­515. 29 Joutel A, Corpechot C, Ducros A, et al. Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Nature 1996; 383:707­710. 30 Dichgans M. Genetics of ischaemic stroke. Lancet Neurol 2007; 6:149­161. 31 Opherk C, Peters N, Herzog J, et al. Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients. Brain 2004; 127 (Pt 11): 2533­2539. 32 Susac JO. Susac's syndrome. AJNR Am J Neuroradiol 2004; 25:351­ 352. 33 Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29:313­316. 34 Aubart-Cohen F, Klein I, Alexandra JF, et al. Long-term outcome in Susac syndrome. Medicine (Baltimore) 2007; 86:93­102. 35 Calabrese LH, Molloy ES, Huang D, Ransohoff RM. Progressive multifocal leukoencephalopathy in rheumatic diseases: Evolving clinical and pathologic patterns of disease. Arthritis Rheum 2007; 56:2116­2128. This paper underlines the occurrence of PML in rheumatic diseases, and its propensity to occur even in the absence of intense immunosuppressive therapy, particularly in SLE. 36 Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334:494­500. 37 Min L, Zwerling J, Ocava LC, et al. Reversible posterior leukoencephalo pathy in connective tissue diseases. Semin Arthritis Rheum 2006; 35:388­ 395. This work reviews the occurrence of reversible posterior leukoencephalopathy in patients with systemic autoimmune diseases.

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