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Vasculitis and nervous system
Dr. Parag MoonSenior resident,
Dept. of Neurology,GMC, Kota.
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Spectrum of clinicopathological disorders characterized by inflammation of blood vessels, including arteries and veins of varying caliber
Result in variety of clinical neurological manifestations related to ischemic injury of central nervous system (CNS) and peripheral nervous system (PNS).
Definition
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Systemic necrotizing arteritis◦ Polyarteritis nodosa◦ Churg–Strauss syndrome◦ Microscopic polyangiitis
Hypersensitivity vasculitis◦ Henoch–Schonlein purpura◦ Hypocomplementemic vasculitis◦ Cryoglobulinemia
Systemic granulomatous vasculitis◦ Wegener granulomatosis◦ Lymphomatoid granulomatosis◦ Lethal midline granuloma
Giant cell arteritis◦ Temporal arteritis◦ Takayasu arteritis
Connective tissue disorders associated with vasculitis◦ Systemic lupus erythematosus◦ Scleroderma◦ Rheumatoid arthritis◦ Sjogren syndrome◦ Mixed connective tissue disease◦ Behcet disease
Inflammatory diabetic vasculopathy
Isolated peripheral nervous system vasculitis
Granulomatous angiitis of the nervous system
Classification of vasculities that affect the nervous system
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Vasculitis associated with infection◦ Varicella zoster virus◦ Spirochetes◦ Treponema pallidum◦ Borrelia burgdorferi◦ Fungi◦ Rickettsia◦ Bacterial meningitis◦ Mycobacterium tuberculosis◦ HIV-1
Central nervous system vasculitis associated with amphetamine abuse
Paraneoplastic vasculitis
Classification of vasculities that affect the nervous system
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Polyarteritis nodosa Necrotizing arteritis and nodules in small-
and medium-sized vessels of heart, liver, kidney, pancreas, testicles, brain, nerves, skeletal muscles sparing lungs and spleen.
Dominant neurological picture-peripheral neuritis with predilection for legs in 50 %
Three quarters-lesions in arteriae nervorum in post mortem studies.
Clinically apparent brain infarcts-10%
Systemic Necrotizing Arteritis
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Histological lesions-mononuclear cell infiltration, necrosis of internal and external elastic lamina of media, fibrin deposition, perivascular inflammation of adventitia, and intimal proliferation ->narrowing of arterial lumina.
Focal deposition of perivascular connective tissue, vascular necrosis, and denuding of endothelium occur
Followed by vascular thrombosis, ischemia, aneurysm formation, rupture, and hemorrhage.
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Multiple small cortical and subcorticalregions of increased signal-infarcts indistribution of small arteries.
Third, or proliferative-chronic inflammatory cells replacing neutrophils of second phase; evidence of necrosis of media, early intimal proliferation (arrowheads), and fibrosis. Lumen isalmost completely occluded.
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Microscopic polyangiitis Differs from PAN in affliction of small arterioles,
capillaries, and venules of lungs and kidney with necrotizing glomerulonephritis.
Circulating antinuclear cytoplasmic autoantibodies (ANCA), usually myeloperoxidase or perinuclear ANCA (p-ANCA)-80%
Epineurial arteries involvement-polyneuropathy mononeuritis multiplex (MNM) type in 1/4th
Skin nodules and purpura in majority.
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Churg-Strauss syndrome Syndrome of asthma, eosinophilia, extravascular
granulomas, and necrotizing vasculitis of small and medium arteries, arterioles, capillaries, and veins.
Angiitis and extravascular necrotizing granulomas with eosinophilic infiltrates.
Granulomas located near small arteries and veins Characterized by palisading epithelioid
histiocytes arranged around central necrotic zones in which eosinophils predominate.
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Three phases. 1. Prodromal period of constitutional symptoms
(rhinitis and asthma). 2. Peripheral blood and tissue eosinophilia3. Systemic vasculitis
Three fourths have neurological involvement similar to PAN
Typically peripheral neuropathy of MNM type, stroke, and hemorrhage.
Laboratory diagnosis-myeloperoxidase or p-ANCA seropositivity, and tissue biopsy.
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Unique predilection for dermis Inflammatory infiltrates-extravasation of
erythrocytes, pronounced endothelial swelling, andinfiltration by PMN and later mononuclear cells, with resultant fibrosis.
Involves arterioles, capillaries and postcapillary venules.
Resultant nuclear fragments or leukocytoclasia and fibrinoid necrosis (termed leukocytoclastic vasculitis [LCV]) and circulating immune complexes deposit in skin and vasculitic lesions.
Lesions-same stage of evolution.
Hypersensitivity Vasculitis
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Leucocytoclastic vasculitis- Entire vessel and perivascular tissue are infiltrated with polymorphonuclear leukocytes and some chronic inflammatory cells with necrosis and nuclear debris with vascular lumen nearly obliterated
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Drug-related vasculitis 20% of dermal vasculitis cases. Clinical spectrum varies from urticaria,
wheezing, and rhinitis; variable serum sickness to laryngeal edema; and hypotension.
Rash mostly maculopapular or vesicular—less often palpable purpura— along arms and legs
More severe drug reactions-multiple organ involvement.
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Results from focal deposition of immune complexes developed from covalent binding of offending drug or its metabolites with native or foreign proteins to produce hapten molecules.
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Serum sickness Varying degrees of infiltration of arterioles,
capillaries, and venules with interstitial inflammation by PMN cells, eosinophils, and mononuclear cells.
Urticaria followed by erythematous or maculopapular rash, petechiae, palpable purpura, and lymphadenopathy
Accompanied by arthralgia, edema, headache, and lethargy.
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Nervous system-◦ brachial plexus neuritis, ◦ mononeuritis simplex and multiplex,◦ Guillain-Barre´ syndrome, ◦ cranial nerve palsies, ◦ blurring of vision, ◦ retinal and palpebral hemorrhages,◦ meningismus, ◦ stroke,◦ myelopathy.
Immune complex disease
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Henoch-Schonlein purpura Nonthrombocytopenic purpura, arthralgia,
abdominal pain, leukocytoclastic vasculitis of skin lesions
Affected child-fever, headache, anorexia. Palpable purpuric lesions-extensor surfaces
of lower extremities and buttocks Associated with migratory angioneurotic
edema of hands, scalp, face, lower legs, and genitalia.
Deposits of immunoglobulins particularly IgA and C3-kidney and blood vessel walls
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Hypocomplementemic vasculitis Urticaria, migratory arthralgia, and
persistent or intermittent hypocomplementemia.
Sometimes severe angioneurotic edema and life-threatening laryngeal edema
Conjunctivitis, episcleritis, uveitis, mild renal disease, pericarditis, abdominal pain, splenomegaly.
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Pseudotumor cerebri-most common neurological manifestation.
Immunological-binding of IgG antibody to C1q along basement membranes with complement activation.
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Cryoglobulinemia Reversibly precipitate at below 37degree Composed of IgG and IgM, complement,
lipoprotein and antigenic protein moieties.◦ Type I-single monoclonal IgM or IgG antibody◦ Type II-mixed, has monoclonal IgM possessing
activity against polyclonal IgG◦ Type III-mixed polyclonal and non-immunoglobulin
Types I and II-lymphoproliferative diseases, particularly multiple myeloma and Waldenstrom macroglobulinemia.
Type III-infection and collagen vascular diseases
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Four vascular lesions (1) occlusion of small and large vessels in type I or II; (2) bland thrombosis of small arteries and arterioles (3) endothelial swelling, proliferation, and basement
membrane thickening (4) LCV.
Peripheral nerves-chronic axonopathy of large myelinated fibers.
Dermatitis, palpable purpura heralded by a sharp or burning sensation.
PNS and CNS-more common with types II and III.
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Cryoprecipitation-cause of ischemia of arterioles and capillaries due to hyperviscosity and direct plugging of small vessels.
CNS manifestations-vascular occlusion with or without vasculitis.
Peripheral neuropathy-epineurial vasculitis, cryoprecipitate deposition, microvascular ischemia with resultant secondary axonopathy.
Inflammatory cell infiltrate-mainly T cell with lesser numbers of B lymphocytes
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Isolation of HCV RNA in peripheral nerve biopsies-unsuccessful.
Presence of cryoglobulinemia, bone marrow studies, nerve biopsy, studies for HCV, HIV, occult cancer, infection, plasma cell dyscrasia, CVD
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Wegener granulomatosis. Triad-necrotizing granulomatous lesions of
sinuses and lower respiratory tract, systemic necrotizing vasculitis of small arteries and veins, and glomerulonephritis
WG lesions begin as minute foci of granular necrosis and fibrinoid degeneration with PMN cells followed by histiocytes and giant cells along margins of granulomas
Systemic GranulomatousVasculitis
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One fourth- CNS involvement Direct destruction of nerve or brain tissue
by necrotizing granulomas Necrotizing arteritis of cerebral and arteriae
nervorum of peripheral nerves. Multifocal pain, sensory loss, and weakness
due to MNM Circulating c-ANCA directed against
proteinase 3.
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Stroke, intracerebral and subarachnoid hemorrhage, optic neuritis
Contiguous extension from nasal and paranasal sinus cavity granulomas-pseudotumor with exophthalmos, extraocular muscles, optic nerve involvement
Extension through temporal bone-middle ear.
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Muscular artery nearly completely destroyed. A large confluent area of fibrinoiddegradation is surrounded by neutrophils, palisading histiocytes, lymphocytes, plasma cells, and some giant cells
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Lymphomatoid granulomatosis. Malignant lymphoreticular disorder with
strong predilection for CNS. Present with constitutional symptoms and
skin lesions resembling erythema nodosum. Focal neurological-MNM, unilateral cranial
nerve palsies, hemiparesis, ataxia, seizures, spinal and radicular syndromes, myopathy.
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CNS-invasion of unifocal and multifocal necrotizing angiocentric and angiodestructive lesions of small- and medium-sized muscular arteries and their endothelia by masses of T cells, plasma cells, histiocytes, and atypical lymphoreticular cells
Immunoblast formation-cerebrum, brain stem, cerebellar parenchyma, meninges
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Lethal midline granuloma. Relentlessly invasive necrotizing process of
nose and palate that causes destruction of sinuses and all major midline structures of head
Producing grotesque facial mutilation and ultimately death.
CNS complications-direct invasion of orbit and face, jugular vein, and sigmoid and cavernous sinuses leading to vascular thrombosis, sepsis, meningitis.
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Temporal arteritis-older adult whites of either gender and involves medium and large arteries.
Headache, scalp tenderness, thickened nodular and pulseless superficial temporal artery, unilateral visual loss, jaw claudication
Along branches of external carotid artery and arteritis of vertebral and carotid arteries typically at end points of dural investment.
Biopsy of temporal artery
Giant Cell Arteritis
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Takayasu arteritis-aorta and its branches in young Asian women.
Obliterative lesions in large arteries such aorta and its major branches.
Dizziness, syncope, subclavian steal, carotid sinus syndrome, stroke, amaurosis fugax, corneal opacification, cataracts, claudication and gangrene of limbs, chest and abdomen angina.
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A, Early lesion of a arge muscular artery, necrosis, inflammation, and giant cell formation (single arrow) seen immediately adjacent to internal elastic lamina (arrowhead), which is undergoing degenerative changes, some intimal proliferation (double arrows) B, More advanced lesion-complete segmental destruction of internal elastic lamina and virtually the entire media (arrows). Marked intimal proliferation has nearly occluded lumen, and few inflammatory cells remain.
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Granulomatous angiitis of the nervous system Nearly exclusive neurological manifestations Headache, mental change, pleocytosis and elevated
protein content in CSF with signs of angiographic beading that precede focal seizure and stroke
Untreated-coma and death. Can occur in association with cell arteritis,
sarcoidosis, varicella-zoster virus, lymphoma, amyloid angiopathy,HIV infection.
Diagnostic biopsy of brain and overlying meninges From named cerebral vessels to medium and small
leptomeningeal vessels
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A, Media and adventitia of this small leptomeningeal artery completely replaced by multinucleated giant cells (arrowheads). Intimal proliferation with obliteration of vascular lumen and a dense, perivascular, mononuclear inflammatory infiltrate B, Larger leptomeningeal vessel-necrosis of media and internal elastic lamina with multinucleated giant cell formation (arrows), intimal proliferation (arrowhead), lymphocytic infiltration of the adventitia and neighboring meninges
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Systemic lupus erythematosus. True vasculitis-present in only 10% Atinuclear antibody (ANA) screen-
homogenous pattern in majority Antibodies to native double- stranded DNA
(anti-dsDNA) and reactivity to the Smith (Sm) and ribonucleoprotein (RNP)
Antiphospholipid antibodies (APA) (lupus anticoagulant (LAC) and anticardiolipin (aCL) antibody)-prothrombotic events
Collagen Vascular Diseases
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Small vessel within brain parenchyma-largely necrotic. Abundant fibrin (darkly stained) is evident in vessel walls and surrounding tissues. A fewchronic inflammatory cells indicate the presence of vasculitis
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Scleroderma. Diffuse fibrosis affecting firstskin and later systemic
organs and nervous system. Vascular lesions-
increased collagen deposition sclerosis, and hyalinization proliferation of endothelium, fibrosis of adventitia and intima,
and duplication and fraying of internal elastic membrane progressive luminal obliteration
Associated with HLA-DQb1 & mediated by three autoantibodies (anticentromere, anti-SCL-70 or topoisomerase, anti-RNA polymerase III)
CREST syndrome
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Digital artery-severe intimal hyperplasia and greater than 90% luminal narrowing. Severe adventitial fibrosis and marked telangiectasia of vasa vasorum is seen, media and internal elastic lamina are relatively spared
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Rheumatoid arthritis. Three forms of vasculitis occur
1. Proliferative endarteritis of few organs, notably heart, skeletal muscle, and nerves characterized by inflammatory infiltration of all layers of small arteries and arterioles, with intimal proliferation, necrosis, and thrombosis.
2. Fulminant vasculitis indistinguishable from PAN3. LCV with palpable purpura, arthritis, cryoglobulinemia,
and low complement levels. Nonvasculitis spinal and epidural involvement-
vertebral collapse, subluxation, and direct narrowing of spinal canal due to rheumatoid pannus.
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Sjogren syndrome Keratoconjunctivitis sicca and xerostomia. Two types of vasculitis
1. LCV of skin with palpable purpura, urticaria, erythematous macules, and papules
2. Resembles PAN with muscle, nerve, CNS, visceral vascular involvement without aneurysm formation.
Associated with extractable RNA proteins Ro or SS-A and intranuclear RNA-associated antigen La or SS-B.
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Mixed connective tissue disease. Clinical and histopathological features of SLE,
scleroderma, and polymyositis Proliferative vascular changes, capillary
involvement, and mild tissue fibrosis. Behcet’s disease. Triad of oral and genital ulcers, uveitis, and
vasculitis Cutaneous, retinal, and CNS vasculitis. Direct inflammation of neuraxis-focal brain stem
meningoencephalitis.
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Purulent bacterial meningitis Mycobacterial Treponema pallidum Borrelia burgdorferi Varicella zoster Fungal-Aspergillus, Candida, Coccidioides,
mucormycosis HIV-associated granulomatous angiitis of
brain, eosinophilic temporal arteritis, vasculitic meningoencephalitis, MNM resembling PAN
Infection-Associated Vasculitis
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Parenteral drug use Necrotizing arteritis of polyarteritis type Amphetamine, cocaine,
phenypropanolamine and opioids, alone or in combination.
Substance Abuse
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Paraneoplastic encephalomyelitis, sensory neuronopathy, high anti-Hu antibody titers
Transmural inflammation of epimysial and epineurial vessels on muscle and nerve biopsy
Mostly seen with cancer of lung, kidney, prostate, lymphoma.
Paraneoplastic Vasculitis
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Blood Studies Complete blood count Erythrocyte sedimentation rate Chemistry panel-creatine phosphokinase Antinuclear antibody Complement levels Rheumatoid factor
Diagnosis-Laboratory investigation
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Cryoglobulins Immunofixation electrophoresis Quantitative immunoglobulins Antibodies (selectively) to: Ro (Sjogren
syndrome [SS]-A), La (SS-B), Smith (Sm), SCL-70, hepatitis B and C virus, human immunodeficiency virus type 1, Borrelia burgdorferi (ELISA, Western blot), c-ANCA, p-ANCA
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Electroencephalography Electromyography and nerve conduction
studies Cerebrospinal fluid analysis: protein,
glucose, cell count, immunoglobulin G level, cytology, VDRL, gram stain, culture, India ink; viral antigens, Lyme Ab, PCR (as indicated)
Neurodiagnostic
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MRI Multiple bilateral cortical and deep white matter
signal abnormalities and enhancement of meninges after gadolinium.
MR angiography-useful in evaluation of medium and large-vessel disease but misses fine-vessel contours
DSA-Beading of vessels-found only in 1/3rd with histologically proven CNS vasculitis, as well as in CNS infection, atherosclerosis, cerebral embolism, and vasospasm of diverse causes.
Multiple microaneurysms-rare in CNS vessels.
Imaging
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Ectasia and beading in M1 segment and lack of flow in the A1 segment of the right anterior cerebral artery (arrow).
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(a) MRangiography showing basilar artery narrowing with irregularity (long arrow) and abrupt cut off of the right vertebral artery (shortarrow)..
(b) Angiogram showing narrowed left internal carotid artery.
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Muscle and nerve biopsy Temporal artery biopsy Meningeal and cortex Skin Systemic organs Lymph nodes
Histopathological
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Immunomodulating agents◦ IvIg
Immunosuppresants◦ Corticisteroids◦ Cyclophosphamide◦ Azathioprine
Treatment
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Physical therapy and orthosis-to maintain range of motion and strength, to improve function status, and to maintain ambulation.
Effective pain management- tricyclic antidepressants, gabapentin, mexiletine, opioids, clonazepam, and topical anesthetic creams
Limit ischemic enhancing effects of other conditions,
such as diabetes mellitus, hypertension and hyperlipidemia
Cessation of cigarette smoking.
Supportive Therapy
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THANK YOU
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NEUROVASCULITIS; Younger, David S;CONTINUUM: Lifelong Learning in Neurology:; Feb 2005:Vol11 - Issue 1:11-42
Central nervous system vasculitis; Rula A. Hajj-Alia, Leonard H. Calabrese: Current Opinion in Rheumatology 2009, 21:10–18
Central nervous system vasculitis:Neil J. Scolding: Semin Immunopathol (2009) 31:527–536
Vasculitis of the nervous system: David S. Younger: Current Opinion in Neurology 2004, 17:317–336
References