dr jameela-approach to a patient with vasculitis

8/3/2019 Dr Jameela-Approach to a Patient With Vasculitis

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GUIDE: DR SANJAY DUBEY

CANDIDATE: DR JAMEELA


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Vasculitis is a clinicopathologic processcharacterized by inflammation and damage to

blood vessels,leading to compromise of thevascular lumen resulating in ischemia of thetissues supplied by the involved vessels.


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PRIMARYVASCULITIS SYNDROMES

PREDOMINANTLY LARGEVESSELVASCULITISGIANT CELL VASCULITISTAKAYASUS ARTERITIS

PREDOMINANTLY MEDIUM VESSELVASCULITISPAN

KAWASAKISDISEASE

PREDOMINANTLY SMALLVESSELVASCULITIS

ANCA +VE---c-ANCA +VE-

WEGENERS GRANULOMATOSIS

p-ANCA +VEMICROSCOPICPOLYANGITISCHURG STRAUSS SYNDROME

ANCA -VEESSENTIAL MIXEDCRYOGLOBULINEMIA

HENOCHSCHONLEINPURPURA

IDIOPATHICCUTANEOUSVASCULITISBECHETS SYNDROME

SECONDARYVASCULITIS SYNDROMES

DRUG INDUCEDVASCULITISHYDRALAZINEPROPYLTHIOURACILALLOPURINOL

THIAZIDES

SERUM SICKNESS

INFECTIONSRICKETTSIAS

SABE

EBVHIV

MALIGNANCIESLYMPHOMAS

CTDsSLE

RASJOGRENSSYNDROME

INFLAMMATORY MYOSITIS

OTHERPRIMARYBILIARYCIRRHOSIS

ULCERATIVECOLITISALPHA 1 ANTIITRYPSINDEFICIENCY

RETROPERITPNEALFIBROSIS


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PATHOGENIC IMMUNE COMPLEX FORMATION AND/OR DEPOSITION

H SPV A S C U L I T I S A / W C O L L A G E N V A S C U L A R D I S E A S E SS E R U M S I C KN E S S & C U TA N E O U S V A S C U L I T I S SY N D R O M E SH EPA T I T I S C A S S O C I A T E D E M C

H EPA T I T I S A S S O S I A T E D PA N

PRODUCTION OF ANTINEUTROPHILIC CYTOPLASMIC ANTIBODIES

W E G E N E RS G R A N U LO M AT O SI SC H U R G S T R A U S S S Y N D R O M E

M I C R O S C OP I C PO L Y A N G I T I S

PATHOGENIC T LYMPHOCYTIC RESPONSES AND GRANULOMA FORMATION

GIANT CELL ARTERITISTAKAYASUS ARTERITS

WEGENERS GRANULOMATOSISCHURG STRAUSS SYNDROME


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Suspect diagnosis and excludeSuspect diagnosis and excludesecondary causessecondary causes

History,History, ClinicalClinical examexam andand LabLab investigationsinvestigations toto detectdetect

generalgeneral featuresfeatures ofof inflammationinflammation andand tissuetissue ischemiaischemia andand totodelineatedelineate organorgan systemssystems involvedinvolved..

Syndrome recognition: recognizeSyndrome recognition: recognize vasculiticvasculiticsyndromes based on clinical findings and lab featuressyndromes based on clinical findings and lab featuresand the patterns of organ system involved.and the patterns of organ system involved.

Confirmation of diagnosisConfirmation of diagnosis: By biopsy and: By biopsy andangiographyangiography

TreatmentTreatment


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Nonspecific systemic symptomsFatigueMalaiseWeaknessFeverAnorexiaWeight loss

Skin involvement: palpable purpura, nodules, ulcers, cutaneous or nailfold infarctions

Musculoskeletal: range from fullblown arthritis to aches in the joints without obvious swelling(arthralgias)

GI: abdominal pain, bleeding

Pulmonary symptoms: cough, dyspnea, hemoptysis

Ocular symptoms: pain, redness, diplopia, visual loss

Cardiac: chest pain, dyspnea

Peripheral nerve symptoms: numbness, weakness, pain consistent with mononeuritis multiplex

CNS symptoms: stroke, transient ischemic attack (TIA)

Renal ds HTN , hematuria


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xConnective tissue disease (SLE, Sjogrenssyndrome, RA,Scleroderma,

Dermatomyositis )xMalignancy (Lymphoma, leukemia)

xTTP

xBronchial asthma

xHIV ds


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Hydralazine Propylthiouracil Allopurinol Thiazides Gold

Sulphonamides Phenytoin penicillin


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Palpable purpura WG, CSS,MPA

Nodules,papules,ulcers,digital ischaemia-PAN

Purpura, papules,vesicobullous lesions-MPA,CSSSkin

Hypertension----

PAN

Kawasakis disease

Bloodpressure

Strawberry tongue,lip cracking,congestion of oro-pharyngeal mucosa-kawasakis ds.

Strawberry gums,gum ulcers-WG

Oral ulcers- hallmark of Bechets disease

Oral cavity


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Seen in any vasculitisPallor

Septal perforation,saddle nose deformity,

mucosal ulceration-WGNose Non purulent conjunctivitis in KDEyes

Lymph nodes U/L cervical lymphadenopathy-KD

Unequal pulse Takayasus arteritis


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b/l cavitory changes are seen in WG

B/l creps feature of interstitial fibrosis-in MPARespiratory system

CHF- seen in CSS

cvs Mononeuritismultiplex-CSS,MPA,WG Visual loss-GCA

Strokes , TIA, -- TAKAYASUS ARTERITISNeurological

Abdominal tenderness- mesentric ischaemia-PAN

GIT Migratory polyarthritis or pauci arthritis-WG,MPA,CSSMusculoskeletal


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Anemia Normocytic Normochromic

Leucocytosis, Thrombocytosis--- Primary Vasculitis

Leukopenia Or Thrombocytopenia ------ Secondary vasculitis-- Like SLE, Malignancy Drug-induced

CBC

Hyperkalemia in the setting of renal failureELECTROLYTES

elevated creatinine in the setting of renalfailureRFT


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may be abnormal in underlying HepatitisB or C infectionLFT

---

RBC casts, hematuria and proteinuria

Sterile pyuria-KDUrinalysis

present in RA, wegeners granulomatosisRheumatoid

factor--

to rule out infectionBlood cultures


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Marked elevationESR , CRP

To screen for SLE, sjogrens syndromeANA --

hypocomplementemia in SLE, CryoglobulinemiaComplements

(C3, C4) --

Rule out hepatitis B or hepatitis C infectionHepatitis B andC serology


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Rule out HIV infectionHIV testing

EMCCryoglobulins

to look for lung involvementCXR


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ROLE OF ANCAANTI NEUTROPHILIC CYTOPLASMIC ANTIBODIES-

DEMONSTRATED BY IMMUNOFLORESCENT STUDY

Abs directed against proteins in cytoplasmic granules of neutrophils andmonocytes

2 types:

c-ANCA against proteinase 3 wegeners granulomatosisSensitivity90% in active ds

-- specifity 95%

p- ANCA against myeloperoxidase

MPA

ChurgStrauss syndrome

WG

ANCA

cardiac invovement2DEchocardiography


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look for aneurysms, stenosis , post

stenotic dilatations in takayyasusarteritis,PAN

Angiography/MRA

Of involved organs/vessels --confirmatory testBiopsy


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WEGENERSGRANULOMATOSIS

Laboratory features C/F

Age- 40 yrs

M:F = 1:1


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CHURG STRAUSSSYNDROME

MEAN AGE OF ONSET 48 YRS

F:M= 1.2:1



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GIANT CELL ARTERITIS

FEMALE PREPONDERANCE

AGE>50 YRS



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C/FLaboratory features

POLYARTERITIS

NODOSA


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FEVER ISTHE MOSTCOMMON CONSTITUTIONALSYMPTOM

KAWASAKIS DISEASE


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Rash in HSP


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SYNDROME IDENTIFICATION IN VASCULITIS SYNDROMES

Unexplained signs and symptoms involving multiple organs

Suspect vasculitis & identify size of vessel involved

Limbclaudication

Assynetric B.P

Absent pulses

bruit

Headache PMR Jaw

claudication Visual loss

Cutanous ulcers, gangrene, nodules

Micro aneurysms Peripheral neuropathy

Post prandial angina

Palpable purpura Vesicobullouslesions Skin granuloma Hamoptysis Mononeuritis multiplex glomerulonephritis

HTN without GN PERIPHRAL

NUROP

ATHY Testicular pain/mass Digital infarcts Deranged RFTS ABSENCE OF URINE

CASTS

Sparing of lung

Oral erythma

Fever

u/l cervical adenopathy

Rash

Coronary aneurysms mucositis

Asthgma / atopy/ nasalallergy/ polypoisis

CHF Mononeuritis multiplex

Palpable purpura Athralgia GN

Abdominal pain/bloodydiarroreh

pulmonary involvement-

alveolar haommorage

Absence of pulmonarynodules

Absence of upper airway ds

Fleeting pulmonary in

filtrates Renal ds in form of RPGN

Hemoptysis GN- HEMATURIA/ RF

Sinusitis /otitis media/saddle nose

Palpable purpura Sub glottic/endobronchial/tracheal

stenosis

LARGE VESSEL-TAKAYASUS

ARTERITIS

LARGE VESSEL-GIANT CELL

ARTERITIS

WEGENERS GRANULOMATOSIS CHURG STRAUSS SYNDROME HSP MICROSCOPIC POLYANGITIS

MEDIUM VESSEL-PANMEDIUM VS---KAWASAKIS

DISEASE

SMALL

VESSEL


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In the active stage of the disease, the

basic instructions include--

Avoiding stress, bed rest, skin care Patients with major pulmonary

involvement,

like in CSS, are advised to avoid

smoking. Antihistamines and NSAIDS reduce

symptoms like pruritus and joint pain.


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Specific therapies are aimed atreducing acute symptoms andpreventing complications.

In cases with underlying disorder, thevasculitic lesions usually resolve

with control of the infectionwithdrawal of the causative drugTreatment of underlying malignancy


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CYCLOPHOSPHAMIDE THERAPYCYCLOPHOSPHAMIDE THERAPY

Indications ANCA +ve vasculitis i.e WG,MPA, CSS with multisystem ds/

lifethreatening ds at presentation

In cases of glucocorticoid failure in CSS, PAN

DOSE ---

2 mg/kg/day orally--- therapy of choice

Or i/v cyclophosphamide intermittent boluses1 gm/m -sq/mnth

Duration--- 3-6 mnths time rqd for remission


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Bone marrow suppression

Cystitis

Bladder cancer

Infertility

GIT intolerance

Opportunistic infections

Pulmonary fibrosis

Myelodysplasia

Teratogenicity

oncogenesis

Management of side effects/ monitoring therapy

Plenty of oral fluids through out day to decrease the risk of b bladder

injury

Monitoring of complete blood count every 1-2 wks and maintaing at >3000/ micro l can prevent cytopenias and decrease risk of infection.


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Methotrexate- indications

Methotrexate induction for non severe disease in WG- ds

isolated to skin joints or sinuses or which is not immediatelylife threatening.

Sygnificant cyclophosphamide toxicity

Maintaining remission

Orally start dose of 0.3 mg/kg single weekly dose max-

15 mg/ wk---------- if well tolerated after 1-2 weeks---increase dose by 2.5 mg

weekly upto 20-25 mg/wk and maintained at this level---- 2 yrs past remission--

- tapered by 2.5 mg each month until discontinued


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ss GIT intolerance Stomatitis

B

one marrow supression hepatoxicity

Pneumonitis

Opportunistic infections

Teratogenicity

To lessen toxicity MTX is given with folic acid1 mg/ day or folinic

acid 5-10 mg once a week

Mtx Is Not To Be Given In Renal Insufficincy Or CLD


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AZATHIOPRINE AND MYCOPHENOLATE MOFETIL

Indications--- Pts who are not able to receive mtx-alternative to mtx

in maintaing remission

DOSE- azathioprine2mg/kg/day

-Mycophenolate mofetil--- 1g BD

No studies comparing mtx or azathioprine-

Choice of agents based on toxicity profile


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Indications for systemic steroid as first-line therapy

Dose 1mg/kg/day---- 1 month with gradualconversion to an alternate day schedule---tapering-----discont after 6 mnths

Severe Ulcerat ive / Necrot ic Cutaneous Lesion

Gastrointest inal B leeding

A c u t e G lo m e ru lo n e p h r i t is Peripheral Neuropathy With Impending Palsy.

Pr im a r y M a n a ge m e n t O f C h u r g St r au s s Sy nd r o me /

G C A / Ta ka ya s u s a r t er i t i s


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s Osteopososis

Cataracts

Glaucoma

Diabetes mellitus

Opprrtunistic infections

Cushingoid features

Hypertension

Myopathy

Peptic ulcer diathesis

Avascular necrosis of bones

Psycosis

Mood alterations

To avoid or decrease side effects-

Attempt to taper corticosteroids to alternate day regime

and discontinue when possible Pts rcving daily glucocorticoids along with cytotoxic drug therapy

should rcvTMP-SMX as Px against P.Jerovici infection


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In hepatitis C virus-associated cases of CV-

--

HBV-associated PAN may need antiviral treatment

Interferon a (IFN a) is the preferred drug.

The treatment schedule consists of 3 million iu of ifn , thrice weekly, fora total duration of 12 to 18 months

Significant improvement (60-80%) in cutaneous, renal and joint

manifestations with decrease in cryoglobulin level

Relapse rate as high as 90%. Ribavirin, with / without IFN --used for

treatment or prevention of relapse.

-

IFN a2 vidarabine lamivudine in combination with plasma exchange.


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Indications

Kawasakis disease---

T/t of choice ---- 2g/kg single dose infusion over 10 hrs in

combination with high dose aspirin

Early administration of IVIg G prevents future risk of aneurysm

formation

Heinoch-schonlein-purpura

It improves cutaneous and systemic involvement (GIT/renal) in pts

with HSP


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Indications

KAWASAKIS DISEASE

High dose aspirin 100mg/kg/day for 14 days f/b 3-5 mg/kg/day for

several weeks in combination with IVig--- reducing coronary

abnormalities.

TEMPORAL ARTERITIS

Aspirin reduces cranial ischaemic compliations in GCA.


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TAKAYASUS ARTERITIS--aggressive surgical /Angioplasty

of stenosed vessels

Reduces risk of stroke

Correcting HTN due to renal artery stenosis

Improves blood flow to viscera and limbs

Leading to decrease mortality and morbidty


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Management of complicationsManagement of complications

y Antihypertensive therapy for HTN

y Angioplasty in Takayasus arteritis

y Management of underlying visceralcomplications- bowel ischaemia,strokes, MI,CHF.

y Renal transplantation in setting ofESRD.


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THANK YOU

dr jameela-approach to a patient with vasculitis

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