05/02/2023
GOOD MORNING GREETINGS FROM
SHARJAH
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SYSTEMIC VASCULITIS
AN OVERVIEW
DR.K.V.RISHIKESAN MD,Dip.NBSPECIALIST PHYSICIAN
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LEARNING OBJECTIVES
•DEFINE VASCULITIS•DEFINE TYPICAL FEATURES S/o VASCULITIS•DESCRIBE THE MODERN CLASSIFICATION SYSTEM • IDENTIFY A PROBABLE VASCULITIS IN A PATIENT WITH A TYPICAL PRESENTATION• TREATMENT OVERVIEW and finally• TAKE HOME MESSAGE
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VASCULITIS
A heterogeneous group of clinical syndromes characterized by inflammation and necrosis of blood vessels
Normal ArteryArtery: WBC in media and
adventitia
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WHAT IS VASCULITIS?
VASCULITIS IS A DIVERSE CATEGORY OF INFLAMMATORY DISEASES OF BLOOD VESSELS.• RANGE IN SEVERITY FROM SELF LIMITED DERMATOLOGIC CONDITIONS TO ACUTE AND RAPIDLY FATAL MULTISYSTEM DISEASES.• ALL FORMS OF VASCULITIS ARE CHARECTERISED BY ENDOTHELIAL DAMAGE, INTIMAL PROLIFERATION, THROMBOSIS AND EVENTUAL VASCULAR OCCLUSION.• VASCULITIS CAN AFFECT EVERY ORGAN SYSTEM.
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WHAT IS VASCULITIS?VASCULITIS IS A DIVERSE CATEGORY OF INFLAMMATORY DISEASES OF BLOOD VESSELS.• RANGE IN SEVERITY FROM SELF
LIMITED DERMATOLOGIC CONDITIONS TO ACUTE AND RAPIDLY FATAL MULTISYSTEM DISEASES.
• ALL FORMS OF VASCULITIS ARE CHARECTERISED BY ENDOTHELIAL DAMAGE, INTIMAL PROLIFERATION, THROMBOSIS AND EVENTUAL VASCULAR OCCLUSION.
• VASCULITIS CAN AFFECT EVERY ORGAN SYSTEM.
Can be difficult to
diagnose and may be life-threatening
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FIBRINOID NECROSISFibrinoid necrosis is a form of necrosis, or tissue death, in which there is accumulation of amorphous, basic, proteinaceous material in the tissue matrix with a staining pattern reminiscent of fibrin.
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SystemicFever, sweats, weight loss
SkinPalpable Purpura
NeurologicMononeuritis Multiplex
MusculoskeletalArthralgia / arthritisMuscle pain / claudication
RespiratorySinusitis / EpistaxisPulmonary infiltrates
Gastrointestinal
Abdominal PainBloody stools
RenalGlomerulo- nephritisHypertension
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WHEN SHOULD VASCULITIS BE SUSPECTED?
• MULTISYSTEM inflammatory disease• Sign. CONSTITUTIONAL SYMPTOMS• RAPIDLY PROGRESSIVE organ dysfunction • HIGH ESRSEVERE anemiaPLATELETS > 500K• Low serum ALBUMIN
CLINICAL FEATURES PARTICULARLY S/Oof small vessel inflammation:
• SKIN: palpable purpura • LUNGS: pulmonary infiltrates /
hemoptysis • KIDNEY: active urinary
sediment• NEURO: foot drop
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TAKE THESE CLUES…..
• Palpable purpura =small vessel leukocytoclastic vasculitis• Hepatitis B PAN• Wrist/foot drop= PAN• Hepatitis C =cryoglobulinemia• Oral & genital ulcers =Behcet’s
Upper/lower airway disease and glomerulonephritis = Wegener’s• Septal perforation, epistaxis• Recurrent sinus infections
Asthma and eosinophilia= CSV
• Young female with arm/leg fatigue and HTN =
Takayasu’s
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Unexplained ischemia, such as claudication, limb ischemia, angina, transient ischemic attack, stroke, mesenteric ischemia and cutaneous ischemia, particularly in a young patient or a patient without risk factors for atherosclerosis
When should vasculitis be suspected?
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CLINICAL FEATURES HIGHLY S/o VASCULITIS
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CUTANEOUS MANIFESTATION
Palpable Purpura
Caused by extravasation of RBCs from small vessels that have been occluded by immunecomplexes. They are heavy and tend to occur in the most gravity dependent area of the body
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LIVEDO RETICULARIS
All of us develop this “lacy” vascular pattern when we are cold. However livedo reticularis is caused by relative ischemia of the capillary beds. Vasculitis is one of the causes of livedo.
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SPLINTER HEMORRHAGES
Splinter hemorrhages can be found in the distal nail bed and/or in the periungual area.
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NEUROLOGICMononeuritis multiplex: check for FOOT DROP
Sural n. biopsy showing vasculitis
Note that the nerve tissue is seen at the bottom of the picture. The vessel infiltration with white blood cells occurs in the vasa nervorum.
nerve tissue
vasa nervorum
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RESPIRATORY: UPPER/LOWER•SINUSITIS• EPISTAXISOr……..
.Pulmonary infiltrates•Nodules•Cavities
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GENITOURINARY•Glomerulonephritis•Hypertension•Hematuria•RBC casts• Testicular pain(especially PAN)
Cellular and Fibrous Crescent Formation in glomerulonephritis. Testicular pain is uncommon in systemic vasculitis, but when it occurs, it is relatively specific for PAN.
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OCULAR
http://www.uveitis.org/images/sarcoid6.jpg
Retinal Vasculitis
http://webmedia.unmc.edu/eye/iritis.jpg
Iritis
Scleritis
eyelearn.med.utoronto.ca/.../RedEye/10Sclera.htm
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GASTROINTESTINAL
• Mesenteric ischemia which may result in :• pain 30 minutes after eating• bloody diarrhea• bowel perforation
• hepatitis• pancreatitis• cholecystitis
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CLASSIFICATIONCONFUSING and UNSATISFYING DUE TO1.OVERLAPPING PRESENTATIONS2.MIX OF PRIMARY AND SECONDARY FORMS3.EVOLVING UNDERSTANDING OF PATHOPHYSIOLOGY4.EVOLVING CONSENSUS REGARDING USE OF EPONYMS5.VARIED OPINION ON WHETHER CLASSIFCATION SHOULD BE BASED PRIMARILY ON SIZE OF AFFECTED VESSELS,OR ON UNDERLYING PATHOLOGIC PROCESS.6.DIFFERENT SOURCES MAY PRESENT SLIGHTLY DIFFERENT CLASSIFICATION SCHEMES
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CLASSIFICATION
No universally accepted classification system.• Vessel size• Histopathology• Dominant organ
involvement. The clinical features of primary vasculitis syndromes often OVERLAP, and many patients do not fit neatly into a well-defined type of vasculitis
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CHAPEL HILL CLASSIFICATION
Large vessels : Aorta and its major branches and the analogous veins. Medium vessels: The main visceral arteries , veins and their initial branches. Small vessels : Intraparenchymal arteries, arterioles, capillaries, venules, and veins. The kidney is used to exemplify medium and small vessels.
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CLASSIFICATION BASED ON Vs.SIZE
• LARGE Vs: GIANT CELL ARTERITIS, TAKAYASU ARTERITIS.
• MEDIUM Vs: PAN, KAWASAKI DISEASE
• SMALL Vs: ANCA POSITIVE MICROSCOPIC
POLYANGIITIS WEGENERS, CHURG
STRAUSS ANCA NEGATIVE
HENOCH SCHONLEIN PURPURA , MIXED ESSENTIAL
CRYOGLOBULINAMIA
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CLASSIFICATIONSOME TYPES PREVIOUSLY HAD DIFFERENT
EPONYM BASED NAMES
OLD NAME • WEGENER’S GRANULOMATOSIS
(WG)• CHURG STRAUSS VASCULITIS (CSV)
• HENOCH SCHOENLEIN PURPURA
NEW NAMEGRANULOMATOUS POLYANGIITIS(GPA)EOSINOPHILIC GRANULOMATOSIS WITH POLY ANGIITIS (EGPA)
IgA VASCULITIS
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GENERAL APPROACH TO VASCULITIS
HISTORY: PATIENT’S STORY
PHYSICAL EXAM: BODY’S STORY
LABORATORY: BEHIND THE-SCENES STORY
Basic CBC, CHEMS, LFTs, UA/micro, CXR
……..MORE PROBLEMS
CREATE PROBLEM LIST………
LIST EVERYTHING [split don’t lump]
PRIORITIZE PROBLEM LIST………
WORK the PROBLEM LIST
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LABS•Determine organ involvement• Exclude other diseases•Routine labs: CBC, BMP, UA, ESR, CRP, LFTs• Infection : cultures, viral serologies (HBV, HCV, HIV)•Autoimmune serologies:ANA, RF, ANCAs, ENA, ds- DNA, C3/C4•Misc: CK, anti-GBM, SPEP, Cryoglobulins
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VASCULITIS MIMICS
•Systemic illness – must exclude alternative diagnoses:•Sepsis•Drug toxicity•Malignancy•Coagulopathy
• INFECTIOUS DISEASES• Endocarditis• HIV
• DRUGS• Cocaine• Methamphetamine
• CHOLESTEROL EMBOLI• ANTIPHOSPHOLIPID ANTIBODY SYNDROME
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APPROACH TO DIAGNOSIS: ANCAs •Antibodies directed against neutrophil granule constituents• c-ANCA•Stains cytoplasm (hence “c”)•Main target antigen: proteinase-3•Highly specific (>90%) for Wegener’s
•p-ANCA•Stains perinuclear (hence “p”)•Main target antigen: myeloperoxidase•A/w MPA and Churg-Strauss
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VASCULITIS: ADDITIONAL TESTING
Imaging studiesSinus CT scanChest CT scanMesenteric angiogramTissue biopsyTemporal artery Sural nerve Muscle Lung Renal
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TISSUE BIOPSY “GO WHERE THE MONEY IS”
Blind biopsy generally low yield of < 20%.Vasculitic lesions tend to be focal and segmental.If a patient is over age 50 Y. and presents with a new, unexplained headache and elevated ESR, with or without a tender or abnormal temporal artery, a temporal artery biopsy would be indicated.
Similarly, in a patient who presents with a multisystem illness and testicular pain and swelling, a testicular biopsy should be considered. Sural nerve biopsy may be indicated in a patient with numbness and tingling in a lower extremity.If the urine sediment is abnormal, a renal biopsy might be obtained.
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ANGIOGRAM
• If biopsy is impractical• Important in large vessel vasculitis• Patient with abdominal pain• Renal or mesenteric vasculitisIf visceral involvement is suspected (and in the absence of a surgical abdomen),
a visceral angiogram to include the celiac, mesenteric, renal and, perhaps, hepatic arteries should be performed .
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TEMPORAL ARTERITIS(GCA)
Anaemia of chronic disease.Blindness (ischemic optic neuropathy) .Claudication jaw/tongueDramatic response to steroidsESR high
Fever, wt. lossHead ache new onset.Scalp tendernessPMR symptoms (proximal muscle pain)10% with large vessel involvement (e.g. subclavian artery)
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GIANT CELL ARTERITISACR CRITERIA (3 OF 5)
• Age > 50• New onset headache• ESR (Westergren) 50 • Abnormal artery biopsy (mononuclear cell infiltrate, granulomatous inflammation, usually multinucleated giant cells)• Temporal artery abnormality (tender or decreased pulse)
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GCA: THERAPY
• Corticosteroids mainstay of therapy (~1 mg/kg)• Calcium and vitamin D• Consider bisphosphonate
• Try to prevent visual loss with therapy:Treat, then biopsy!Necrosis of Intima and media with disruption of Internal Elastic Lamina
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QUESTIONS
• In the ACR diagnostic criteria for Giant Cell Arteritis (Temporal Arteritis), a patient needs to be greater than what age?A. > 40 yearsB. > 50 yearsC. > 60 yearsD. > 70 yearsE. > 80 years
B. > 50 YEARSAlmost all are >
60Average age is 70
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TAKAYASU’S• Large vessel• Unknown etiology• Aorta/branches• “Pulseless Disease”• Women in reproductive years
• 10X more than men• Asia, Eastern Europe, Latin America
• Granulomatous Panarteritis: 98% have stenotic lesions, 27% aneurysms
• Subclavian & aortic arch most common, 93%
• 40- 80% renal artery stenosis
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TAKAYASU’S• Arterial stenoses/organ ischemia
• Claudication• Transient cerebral ischemia/ stroke• Renal artery hypertension• CHF• Angina• MI• Mesenteric vascular insufficiency
• In the absence of complications (retinopathy, HTN, aortic v. insuff), 15 yr survival 95%
• Most respond to steroids alone• 40% will need cytotoxics
Aortogram of a 15-year-old girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery.
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POLY ARTERITIS NODOSA
• Necrotizing vasculitis of medium & small arteries• Age ~ 40s; M > F• Constitutional symptoms are common• fever 50%• weight loss50%
• Vasculitis can be variable in distribution making diagnosis difficult
• Mononeuritis multiplex:50%• Renal involvement: 60% (renal arteries, interlobular
arteries)• Hypertension (more common)• Glomerulonephritis (uncommon)
• Abdominal involvement 45%• Arthralgias/ Myalgias / Myositis
64%• Testicular pain 25%•Pulmonary involvement rare
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CASE• A 29yo woman presents with a
4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. Treatment has mostly focused on wound care.
• On exam the ulcers are noted, as is livedo reticularis, a decreased right hand grip, and a right foot drop.
• Which of the following is the most likely diagnosis:
A. LYMPHOMA WITH A PARANEOPLASTIC SYNDROME
B. TAKAYASU’S ARTERITIS
C. SLE
D. PAN
E. KAWASAKI DISEASE
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PAN : CLINICAL FEATURESMYALGIA, ARTHRALGIAFEVER , WT LOSSPALPABLE PURPURIC RASH, ULCERATION, INFARCTION AND LIVEDO RETICULARISARTERITIS OF VASA NERVORUM LEADS TO A SYMMETRICAL SENSORY AND MOTOR NEUROPATHY.RENAL INFARCTIONS MAY LEAD TO SEVERE HTN AND RENAL IMPAIRMENT.
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POLY ARTERITIS NODOSAHepatitis B (surface antigen) is risk factor for PAN.Classic PAN is NOT associated with ANCA
ANCA
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KAWASAKI DISEASE
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KD : THE MUCOCUTANEOUS LYMPH NODE SYNDROME
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KAWASAKI DISEASE
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KAWASAKI DISEASE : F E B Ri L E
Kawasaki disease (KD) is an acute febrile vasculitic syndrome of early childhood that, (although it has a good prognosis with treatment) can lead to death from coronary artery aneurysm (CAA) in a very small percentage of patients.
CRITERIA • Fever • Enanthem • Bulbar conjunctivitis • Rash • Internal organ involvement (not part of the criteria) • Lymphadenopathy • Extremity changes
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ANCA ASSOCIATED VASCULITIS• They have been classified together in the recent 2012 Chapel Hill classification system.• GPA and MPA have been considered together in clinical and treatment studies because they share many clinical features. • Renal involvement is identical in both diseases with a pauci immune FSGN. Granulomatous involvement, of the upper respiratory tract, is a characteristic feature of GPA, but not of MPA• Eosinophilia, asthma and atopy typically occur in EGPA
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TYPES OF ANCA ASSOCIATED VASCULITIDES
There are 3 forms of ANCA-associated vasculitis. • Granulomatosis with
polyangiitis (GPA) • Formerly known as
Wegener's granulomatosis• Microscopic polyangiitis (MPA)• Eosinophilic granulomatosis
with polyangiitis (EGPA) • Formerly known as Churg-
Strauss syndrome
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WEGENER’S GRANULOMATOSIS (GPA)
• Necrotizing vasculitis that affects the small vessels of the respiratory tract and renal system : PULMONARY-RENAL SYNDROME
• Age ~ 40s: M > F 2:1• Sinusitis
• Nasal septal ulceration• Pneumonitis
• few symptoms until late• usually no mediastinal
lymphadenopathy• nodules that can cavitate
ACR CRITERIA (3 OUT OF 5)1.Nasal or oral inflammation (oral ulcers or bloody nasal drainage)2.Abnormal chest radiograph (nodules, fixed infiltrates, cavities)3.Urinary sediment (> 5 RBC/ hpf or casts)4.Abnormal Biopsy: showing vasculitis5.Proteinase-3 antibodies
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WEGENER’S GRANULOMATOSIS (GPA)
A 65yo. woman c/o 6 months of malaise, 9 lb weight loss, recurrent sinusitis, and a persistent cough. On exam, she is afebrile, the mid-portion of the nasal bridge has a flattened appearance, and both sides of the nasal septum are ulcerated. RF is positive and ESR is 66.
• Which of the following tests would be most helpful in determining the diagnosis:A. Nasal septum biopsyB. Chest radiographC. Measurement of ANCA antibodiesD. Sputum cultureE. Measurement of anti-GBM antibodies
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GRANULOMATOUS POLYANGIITISWEGENER’S : ANCA
• AntiNeutrophil Cytoplasmic Antibody• C (cytoplasmic staining) ANCA• Proteinase 3 (C is the 3rd letter)
• Pulmonary-renal disease• sensitivity of 95%• specificity of 95%
• Limited disease…• lower sensitivity and specificity
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GRANULOMATOUS POLYANGIITIS(GPA) : RX
• Prior to cyclophosphamide, 80-90% mortality at 5 years• With cyclophosphamide, 5-10% mortality• Concern about long-term toxicity of PO cyclophosphamide (bladder especially)• IV CYTOXAN no significant bladder risk• Rituximab: very effective for induction & maintenance• Azathioprine for maintenance
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WEGENER’S GRANULOMATOSIS: OUTCOMES
INTERVENTION SURVIVAL
NONE 50% AT 5 MONTHS
GLUCOCORTICOIDS 50% AT 1 YEAR
GCS+CYTOXAN 80% AT 8 YEARS
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CHURG-STRAUSS VASCULITIS (EGPA)
• Necrotizing, granulomatous vasculitis of small arteries and venules• Prior asthma• Allergic rhinitis• Eosinophilia• Pulmonary infiltrates.Classical presentation as a triad of skin nodules, mononeuritis multiplex and eosinophilia
• Intra/extravascular granulomas• Confusion with Wegener’s• Nasal/sinus DZ is NON-destructive• Pulmonary nodules less common
• p-ANCA (MPO): 70%• More responsive to steroids alone
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CHURG STRAUSS
THE BASTARD CHILD OF WEGENER’S AND POLYARTERITIS NODOSA.CSS SHARES FEATURES OF BOTH WEGENER’S AND PAN.PATIENT WITH CSS ALWAYS HAS ASTHMA
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CHURG STRAUSS VASCULITIS(EGPA)
WEGENERS(WG)
ASTHMA +++ UNCOMMON
EOSINOPHILS +++ OCCASIONAL/MODEST
ATOPY +++ UNCOMMON
UPPER AIRWAY DESTRUCTION : UNCOMMON
+
PULMONARY NODULES: OCCASIONAL
++
RENAL FAILURE : + ++
ANCA + +
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MICROSCOPIC POLYANGIITIS (MPA)
• CLASSICALLY PRESENTS WITH RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS OFTEN ASSOCIATED WITH PULMONARY ALVEOLAR HAEMORRHAGE.• CUTANEOUS INVOLVEMENT SIMILAR TO PAN IS COMMON• PATIENTS ARE USUALLY p-ANCA POSITIVE
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CASE: WHAT IS THE MOST LIKELY CAUSE OF RENAL DISEASE IN THIS PATIENT ?
• A previously healthy 22yo. male college student had an URI 2 weeks ago, RX with PCN• He develops abdominal pain, bilateral ankle pain & swelling with raised purpuric lesions over lower extremities• Labs:• creatinine 3.0 mg/d L, BUN 46 mg/dL• Urinalysis: 4+ proteinuria, 2+ RBC’s, • sev. RBC casts/ hpf
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HSP MANIFESTATIONS
Renal involvement (10-50%)• Renal disease more severe in adults• Determines prognosis• Many recover with no therapy• Asymptomatic hematuria proteinuria & renal insufficiency (cresentic GN)• < 0.5% progress to ESRD
Small vessels, post capillary venules Palpable purpura, Arthralgias,GI INVOLVEMENT IN 85% Abdominal pain, N&V,hemorrhageIntussusception in children as the major life threatening complicationMales=femalesMean age 5 yrs.Preceding URI in 2/3 (1-3 weeks)Tissue deposition of IgA-containing immune complexes (skin, kidneys, bowel)
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HSP: IgA VASCULITISUsually single episodes < 4 weeks duration.Glomerulonephritis indistinguishable from IgA nephropathy may occur. Uncommon: #Testicular involvement
#Pulmonary hemorrhage#CNS complications
40% recurrence rate after period of wellnessTreatment•Supportive measures•Corticosteroids for GI vasculitis and hemorrhage• ? CS early in nephritis
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CRYOGLOBULINAEMIA
PATTERN OF ORGAN INVOLVEMENT:• Constitutional• Cutaneous• Raynaud’s• Articular• Vascular• Neurologic
Rheumatoid factor Complement C4 ↓ ↓ ↓Cryoglobulin (cryocrit)
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CRYOGLOBULINAEMIA
An immune-complex-mediated small vessels Vasculitis.A causative role of hepatitis C virus in over 80% patients .Cryoprecipitate Hep C Ag – AbComplement fixing: C4 consumptionC4 levels VERY low
Therapeutic strategies include Aetiologic (antiviral), Pathogenetic (cyclophosfamide, rituximab), or Symptomatic (steroids, plasmapheresis)
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BEHCET DISEASE
Behçet disease:A rare vasculitic disorder.Characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitisAUTOIMMUNE DISEASE.The pathergy test is helpful but is not sensitive or specific for the diagnosis of Behcet disease.
Oral aphthous ulcers secondary to Behçet
disease.
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RARE VASCULITIS WHICH TARGETS VENULES. TREATMENT: CORTICOSTEROIDS,THALIDOMIDE, IMMUNOSUPPRESSIVE
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VASCULITIS: PRINCIPLES OF TREATMENT
ORAL GLUCOCORTICOIDS IV METHYL PREDNISOLONE METHOTREXATE AZATHIOPRINE CYCLOSPORINE RITUXIMAB-ANTI CD20AbsANTI TNF THERAPIES INFLIXIMAB ADALIMUMAB ETANERCEPT CERTULIZUMAB
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CHOICE OF THERAPY…….DEPENDS ON : SEVERITY OF ORGAN DAMAGE EXTEND OF MULTISYSTEM INVOLVEMENT THE VASCULAR BED INVOLVED:(RENAL, OCCULAR,CORONARY.)GLUCOCORTICOIDS + CYCLOPHOSPHAMIDE PREFERREDFOR SERIOUS AND SEVERE COMPLICATIONS.GLUCOCORTICOIDS ALONE WILL SUFFICE FOR ISOLATED MILD VASCULITIS LIKE IDIOPATHIC CUTANEOUS VASCULITIS
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TREATMENT OVERVIEW
LIMITED CUTANEOUS VASCULITIS OBSERVATION… ANTIHISTAMINE…. SHORT COURSE PREDNISONE. MILD SYSTEMIC VASCULITIS PREDNISONE
RAPIDLY PROGRESSIVE VASCULITISPREDNISONE+ CYTOTOXIC (CYCLOPHOSPHAMIDE).CYC. TRANSITIONED TO LESS TOXIC AGENTS (AZA, METHOTREXATE) AFTER REMISSION IS ACHIEVED.
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VASCULITIS: ACTIVITY Vs. DAMAGES
Disease Activity
Disease Damage
Even after achieving disease remission, patients will continue to suffer from the chronic, irreversible consequences of both the disease and its therapies
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TAKE HOME MESSAGE• The systemic vasculitides are chronic diseases, usually autoimmune , characterized by relapse and remission• Ultimate diagnosis is almost always biopsy• Achieving remission requires intense monitoring by an expert multidisciplinary team • Therapy of choice usually involves corticosteroids and sometimes an adjunctive cytotoxic• Pain and fatigue are common consequences independent of disease activity and generally fail to respond to immunosuppression
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Questions?