05/02/2023

GOOD MORNING GREETINGS FROM

SHARJAH

05/02/2023

SYSTEMIC VASCULITIS

AN OVERVIEW

DR.K.V.RISHIKESAN MD,Dip.NBSPECIALIST PHYSICIAN

05/02/2023

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

05/02/2023

VASCULITIS

A heterogeneous group of clinical syndromes characterized by inflammation and necrosis of blood vessels

Normal ArteryArtery: WBC in media and

adventitia

05/02/2023

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.

05/02/2023

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

05/02/2023

05/02/2023

05/02/2023

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.

05/02/2023

SystemicFever, sweats, weight loss

SkinPalpable Purpura

NeurologicMononeuritis Multiplex

MusculoskeletalArthralgia / arthritisMuscle pain / claudication

RespiratorySinusitis / EpistaxisPulmonary infiltrates

Gastrointestinal

Abdominal PainBloody stools

RenalGlomerulo- nephritisHypertension

05/02/2023

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

05/02/2023

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

05/02/2023

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?

05/02/2023

CLINICAL FEATURES HIGHLY S/o VASCULITIS

05/02/2023

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

05/02/2023

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.

05/02/2023

SPLINTER HEMORRHAGES

Splinter hemorrhages can be found in the distal nail bed and/or in the periungual area.

05/02/2023

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

05/02/2023

RESPIRATORY: UPPER/LOWER•SINUSITIS• EPISTAXISOr……..

.Pulmonary infiltrates•Nodules•Cavities

05/02/2023

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.

05/02/2023

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

05/02/2023

GASTROINTESTINAL

• Mesenteric ischemia which may result in :• pain 30 minutes after eating• bloody diarrhea• bowel perforation

• hepatitis• pancreatitis• cholecystitis

05/02/2023

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

05/02/2023

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

05/02/2023

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.

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

VASCULITIS: ADDITIONAL TESTING

Imaging studiesSinus CT scanChest CT scanMesenteric angiogramTissue biopsyTemporal artery Sural nerve Muscle Lung Renal

05/02/2023

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.

05/02/2023

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 .

05/02/2023

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)

05/02/2023

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)

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

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.

05/02/2023

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

05/02/2023

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

05/02/2023

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.

05/02/2023

POLY ARTERITIS NODOSAHepatitis B (surface antigen) is risk factor for PAN.Classic PAN is NOT associated with ANCA

ANCA

05/02/2023

KAWASAKI DISEASE

05/02/2023

KD : THE MUCOCUTANEOUS LYMPH NODE SYNDROME

05/02/2023

KAWASAKI DISEASE

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

05/02/2023

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

05/02/2023

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

05/02/2023

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

05/02/2023

WEGENER’S GRANULOMATOSIS: OUTCOMES

INTERVENTION SURVIVAL

NONE 50% AT 5 MONTHS

GLUCOCORTICOIDS 50% AT 1 YEAR

GCS+CYTOXAN 80% AT 8 YEARS

05/02/2023

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

05/02/2023

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

05/02/2023

05/02/2023

CHURG STRAUSS VASCULITIS(EGPA)

WEGENERS(WG)

ASTHMA +++ UNCOMMON

EOSINOPHILS +++ OCCASIONAL/MODEST

ATOPY +++ UNCOMMON

UPPER AIRWAY DESTRUCTION : UNCOMMON

+

PULMONARY NODULES: OCCASIONAL

++

RENAL FAILURE : + ++

ANCA + +

05/02/2023

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

05/02/2023

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

05/02/2023

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)

05/02/2023

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

05/02/2023

CRYOGLOBULINAEMIA

PATTERN OF ORGAN INVOLVEMENT:• Constitutional• Cutaneous• Raynaud’s• Articular• Vascular• Neurologic

Rheumatoid factor Complement C4 ↓ ↓ ↓Cryoglobulin (cryocrit)

05/02/2023

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)

05/02/2023

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.

05/02/2023

RARE VASCULITIS WHICH TARGETS VENULES. TREATMENT: CORTICOSTEROIDS,THALIDOMIDE, IMMUNOSUPPRESSIVE

05/02/2023

VASCULITIS: PRINCIPLES OF TREATMENT

ORAL GLUCOCORTICOIDS IV METHYL PREDNISOLONE METHOTREXATE AZATHIOPRINE CYCLOSPORINE RITUXIMAB-ANTI CD20AbsANTI TNF THERAPIES INFLIXIMAB ADALIMUMAB ETANERCEPT CERTULIZUMAB

05/02/2023

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

05/02/2023

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.

05/02/2023

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

05/02/2023

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

05/02/2023

Questions?