VASCULITIS SYNDROMESVASCULITIS SYNDROMES

Mohammad Reza Shakibi M.DKerman university of medical sciences (KMU)y ( )Shafa Hospital, Rheumatology ward

ILLUSTRATED CASE 1 ILLUSTRATED CASE 1

A 56 years old lady refered me for prolonged fever,arthritis and burning pain in her foots and hands.She had history of transfusion during operation 5years ago and HBV infection.Sh h d hi t f 3 th f f tig l fShe had history of 3 month fever, fatigue, loss ofappetite, postprandial abdominal pain and 5 Kgweight loss during this time She also had arthritisweight loss during this time. She also had arthritisin hand joints and severe burning pain in footsand hands. She noticed inability to extent left wrist.

ILLUSTRATED CASE 1 (CONTINUE)ILLUSTRATED CASE 1 (CONTINUE)

Her Ph/Exam showed :Her Ph/Exam showed :Fever, arthritis, wrist drop,

gangrene in finger tips .g g g p

ILLUSTRATED CASE 1 (CONTINUE)ILLUSTRATED CASE 1 (CONTINUE)

She diagnosed vasculitis and treatment She diagnosed vasculitis and treatment started.

Two days later she had sever abdominal pain Two days later she had sever abdominal pain and operation showed massive intestinal gangrene gangrene.

She die 2 days later.

WHAT IS VASCULITIS?WHAT IS VASCULITIS?Vasculitis is a clinicopathologic process characterized by inflammation of and damage to blood vesselsinflammation of and damage to blood vessels.The vessel lumen is usually compromised, and this is associated with ischemia of the tissues supplied by the involved vesselinvolved vessel.A broad and heterogeneous group of syndromes may result from this process, since any type, size, and l i f bl d l b i l d location of blood vessel may be involved. Vasculitis and its consequences may be the primary or sole manifestation of a disease; alternatively, vasculitis; y,may be a secondary component of another primary disease. Vasculitis may be confined to a single organ such as the Vasculitis may be confined to a single organ, such as the skin, or it may simultaneously involve several organ systems.

POTENTIAL MECHANISMS OF VESSEL DAMAGE IN VASCULITIS SYNDROMESIN VASCULITIS SYNDROMES

Pathogenic immune complex formation and/or depositionH h S hö l iHenoch-Schönlein purpuraVasculitis associated with collagen vascular diseasesSerum sickness and cutaneous vasculitis syndromesHepatitis C–associated essential mixed cryoglobulinemiaHepatitis B–associated polyarteritis nodosa

Production of antineutrophilic cytoplasmic antibodiesWegener's granulomatosisChurg-Strauss syndromeMicroscopic polyangiitis

Pathogenic T lymphocyte responses and granuloma formationGiant cell arteritisTakayasu's arteritisyWegener's granulomatosisChurg-Strauss syndrome

VASCULITIS CLINICAL MANIFESTATIONSVASCULITIS CLINICAL MANIFESTATIONS

Systemic symptoms Systemic symptoms Constitutional symptoms: fever, weight loss, malaise, arthralgias/arthritis, loss of appetite, malaise, arthralgias/arthritis, loss of appetite, generals body pain

Localized symptoms (According vessel size )Localized symptoms (According vessel size )Large Vessel VasculitidesMedium Vessel VasculitidesMedium Vessel VasculitidesSmall Vessel Vasculitides

Vasculitis Clinical manifestationsLarge Vessel Vasculitides

Limb claudicationLimb claudicationAsymmetric blood pressuresAb f lAbsence of pulsesBruitsAortic dilationRenovascular hypertensionRenovascular hypertension

Vasculitis Clinical manifestationsLarge Vessel Vasculitides

Takayasu arteritis (TAK)Takayasu arteritis (TAK)Giant cell arteritis (GCA)

Vasculitis Clinical manifestationsMedium Vessel Vasculitides

Cutaneous nodulesCutaneous nodulesUlcersLi d ti l iLivedo reticularisDigital gangreneMononeuritis multiplexMicroaneurysmsMicroaneurysmsRenovascular hypertension

Vasculitis Clinical manifestationsMedium Vessel Vasculitides

Polyarteritis nodosa (PAN)Polyarteritis nodosa (PAN)Kawasaki disease (KD)

Vasculitis Clinical manifestationsSmall Vessel Vasculitides

PurpuraPurpuraVesiculobullous lesionsU ti iUrticariaGlomerulonephritisAlveolar hemorrhageCutaneous extravascular necrotizingCutaneous extravascular necrotizing granulomasSplinter hemorrhagesSplinter hemorrhagesUveitis/episcleritis/scleritis

Vasculitis Clinical manifestationsSmall Vessel Vasculitides

Antineutrophil cytoplasmic antibody (ANCA)–p y p y ( )associated vasculitis (AAV)

Microscopic polyangiitis (MPA)Granulomatosis with polyangiitis (Wegener’s) (GPA)Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)Strauss) (EGPA)

Immune complex SVVAnti glomerular basement membrane (anti GBM) diseaseAnti–glomerular basement membrane (anti-GBM) diseaseCryoglobulinemic vasculitis (CV)IgA vasculitis (Henoch-Scho¨nlein) (IgAV)IgA vasculitis (Henoch Scho nlein) (IgAV)Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

PANCA PATTERN

CANCA PATTERN

DIFFERENTIAL DIAGNOSIS FOR PRIMARY SYSTEMIC VASCULITIS

InfectionEndocarditisSepsisSyphilisMeningococcemiaMeningococcemiaViral hepatitisTuberculosis

Di d f C l tiDisorders of CoagulationDisseminated intravascular coagulationAntiphospholipid antibody syndromep p p y yThrombotic thrombocytopenic purpura

Drug ToxicityS th i ti tSympathomimetic agentsCocaine

DIFFERENTIAL DIAGNOSIS FOR PRIMARY SYSTEMIC VASCULITIS

Atherosclerotic and Embolic DiseasePeripheral vascular diseaseCholesterol embolization syndromeGeneralized atherosclerosisCardiac myxomas

MalignancyLymphomaDi i t d i t iDisseminated carcinomatosis

Secondary Vasculitides Associated with Other Autoimmune Conditions

Systemic lupus erythematosusSjögren syndromeRheumatoid arthritisRheumatoid arthritisBehçet diseaseSarcoidosis

DIFFERENTIAL DIAGNOSIS FOR PRIMARY SYSTEMIC VASCULITIS

Congenital Abnormalities and Anatomic VariantsFibromuscular dysplasiaEhlers-Danlos syndromeEhlers Danlos syndromeMarfan syndromeOth Di dOther DisordersBuerger diseaseCalciphylaxisAmyloidosisAmyloidosis