VASCULITIS SYNDROMES
Transcript of VASCULITIS SYNDROMES
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