Vasculitis syndrome an approach -and-basic principles of treatment
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Transcript of Vasculitis syndrome an approach -and-basic principles of treatment
Vasculitis SyndromeAn Approach
AndBasic Principles of Treatment
Dr. Sachin Verma MD, FICM, FCCS, ICFCFellowship in Intensive Care Medicine
Infection Control Fellows Course Consultant Internal Medicine and Critical Care
Ivy Hospital Sector 71 MohaliWeb:- http://www.medicinedoctorinchandigarh.com
Mob:- +91-7508677495
Introduction• Vasculitides are a hetrogenous group of
conditions characterized by inflammation and necrosis of blood vessels.
• A broad group of syndromes may result from this process,since any type,size, and location of vessel may be involved.
Classification of vasculitides:Various attempts made to create a classification of
vasculitides.
But it remains a matter of controversy.
Most classifications are based on: 1. Size vessels it involves. 2. Histological findings from involved vessels. 3.Combination of both vessel size and histological
findings. But all scheme of classification is imperfect.
Pathophysiology and pathogenesis Pathogenic immune-complex formation: 1.Most widely accepted mechanism 2.Casual role is not clearly established Examples- PAN, EMC Antineutrophilic cytoplasmic antibodies(ANCA): Two types- 1. c-ANCA, Examples; WG 2. p-ANCA,Examples;MPA, CSS, Crecentric
GN, GPS , WG Pathogenic T-Lymphocytes response and granuloma
formation: Examples; WG , Giant cell arteritis,Takayasu arteritis CSS
Etiopathogenic classification incorporating the modified CHCC Classification by Lie
DOMINENT VESSELS
PRIMARY VASCULITIS PATHOGENESIS
LARGE ARTERIES Temporal arteritisTakayasu’s arteritis
T cell mediated
MEDIUM ARTERIES Classical PAN
Kawasaki’s disease
Immune complex related
Antibody mediated hypersensitivity
SMALL AND MEDIUM SIZE ARTERIES
Wegener’s granulomatosisCSSMSA
Antibody mediated (ANCA)
SMALL VESSEL VASCULITIS(Leukocytoclastic)
HSPEMCCUTANEOUS LCV
Immune complex mediated
The most recent classification scheme proposed by the American College of Rheumatology (ACR)
• Uses both vessel size and type of inflammatory infiltrate.• It classifies vasculitis as follows: Polyarteritis nodosa (PAN), Churg-Strauss syndrome, Wegener's granulomatosis, Hypersensitivity vasculitis, Henoch-Schönlein purpura, Giant cell arteritis, Takayasu's arteritis, Granulomatous angitis of CNS, Berger's
disease, and Kawasaki disease
INDIAN PERSPECTIVE• Reliable epidemiological data from india is
not available• Takayasu’s arteritis is commonest
vasculitis described.• Temporal arteritis is extremly uncommon• Patients with WG are seen in significant
number in north,but rare in south.• Classical PAN has been described all over.
Frequency distribution of vasculitic disorders in India (N=1064)*
DISEASE NO. PERCENTAGE
Aortoarteritis 215 20.20Giant cell arteritis 35 3.36
Polyarteritis nodosa (PAN) 95 8.83
Cutaneous PAN 13 1.22
Wegener’s granulomatosis 147 13.83
Microscopic polyangiitis 42 3.94
Churg Straus syndrome 19 1.78
Henoch Schonlein purpura 232 21.80Small vessel vasculitis 61 5.73
Behcet’s disease 145 13.62
Kawasaki disease 05 0.46
Undiagnosed 50 4.69
Others** 6 0.56
CLINICAL MANIFESTATIONS
• The Vasculitides are truly a ‘multisystem’ diseases. No organ or system is spared.
• General Symptomatology : Fever Weight loss Malaise Fatigue Night sweats Anemia Generalised aches and pain
CLINICAL MANIFESTATIONS:CLINICAL MANIFESTATIONS : A GUIDE TO THE TYPE OF VESSELS INVOLVED
LARGE VESSELS MEDIUM VESSELS SMALL VESSELS
Limb claudication Red and blue Panniculitis Purpura
Asymmetric blood pressure Ulcer Vesicobullous lesions
Absence of pulses Livedo reticularis. Urticaria
Bruits Digital gangrene Glomerulonephritis
Aortic dilatation Mononeuritis multiplex Alveolar haemorrhage
Renovascular Hypertension Microaneurysm Splinter haemorrhage
Reno-vascular hypertension Uveitis,episcleritis,scleritis
Mucosal ulcer in bowel
Continue…• SKIN: Skin commonly involved.o Palpable purpura is commonest dermal
lesion.o Other specific lesions are: nodules and plaques which may ulcerate Infarcts Ulcers Pyoderma gangrenosum Wide spread skin necrosis and gangrene. Macules, papules,vesicles, blisters, and small
bullae has been described.
SKIN LESIONS
Muscles and Joints: Features are common, but nonspecific. Arthralgia and Arthritis Generalised Myalgia and Weakness Claudication, PainEye ,Ear ,Nose and Throat: Scleritis Scleromalacia Perforation of globe Uveatis Recurrent Otitis media Hearing loss Recurrent sinusitis, nasal septum damage Several and recurrent oral ulcerations
Airways and Lungs: Stridor Ventilatory compromise Cough Chest pain Expectoration and Hemoptysis Pulmonary lesions like: infiltrate nodules cavities mass lesions abscess
Gastrointestinal tract: most important G.I. manifestations are due to
bowel ischemia.
Kidneys: Great diversity of lesions: Renovascular hypertension Infarction and Hematoma of kidneys Mild GNs to rapidly progressive GNs
Nervous system: Peripheral Neuropathy, Stroke
Reproductive system: Testicular Infarction Scrotal ulcers Penile ulcers on Glans and Shaft
Cardiovascular system : Pericarditis, Myocardial infarction Aneurysmal rupture
Approach to the patient• Diagnosis of vasculitis is considered in an any
patient with unexplained illness.
• Certain clinical abnormalities when present alone or in combination suggest a diagnosis of vasculitis like…
1.Palpable purpura 2.Pulmanary infiltrates 3. Microscopic hematuria 4.Chronic inflammatory sinusitis 5.Unexplained ischemic events 6.Glomerulonephritis with evidence of multisystem disease
Laboratory Work-up• First to exclude the diseases which can
mimic vasculitis.• To establish the category of vasculitis
syndrome. Hemogram : Normocytic Normochromic anemia Leukocytosis(>10% Eosinophils in CSS)Acute phase reactants: Raised ESR, CRP, alpha-2 globulin,
fibrinogen, Thrombocytosis, ALP
Continue….Urine analysis: Hematuria ProteinuriaSerum proteins: Hypergammaglobulinemia (mostly IgG type, IgA in HSP, WG) Complements levels usually decreasedAntineutrophil cytoplasmic
autoantibodies (ANCA): c-ANCA p-ANCA A or x-ANCA
Continue….Organ biopsy: Gold standard for diagnosis of vasculitis.
Angiography : Especially for medium and large vessels. Others: X-rays, CT Scan, MRI of thorax MRA USG Studies
Basic principals of treatment • Glucocorticoids
• Glucocorticoids + cytototoxic drugs
• Antiviral therapy if indicated
• Plasma exchange and IV Ig + or aspirin
• Surgical correction and Angioplasty
Continue…• Sympotomatic mangement in cases of
hypersensitivity vasculitis
• White blood cell counts every 1-2wks and WBC count should be maintained above > 3000/microL.
• TMP-SMX should be given to every patient receiving glucocorticoids and cytotoxic drugs combination therapy.
SMALL AND MEDIUM SIZED
VASCULITIS SYNDROMES
WEGENNER’SGRANULOMATOSIS
CHURG SRAUSS SYNDROME
MICROSCOPICPOLYANGITIS PAN
M:F1:1
1:14(INDIA)
1.2:1NO MUCH DIFF. FROM WESTRN DATA
OVER ALL UNKNOWN
OVER ALL UNKNOWN
4.5:1(INDIA)
TYPE OF VESSELS INVOLVED
SMALL ARTERIES AND VEINS
SMALL AND MEDIUM SIZED VESSELS
SMALL VESSELS(ARTERIES,CAPILLARIES,VENULES)
SMALL AND MEDIUM SIZED ARTERIES ONLY
SPECIFIC FEATURE
TRIAD: UPPER AND LOWER AIR WAYS WITH KIDNEY LESIONS
EXTRA VASCULAR GRANULOMA
PULMONARY CAPILLARIES INVOLVED,GNs +NT
PULMONARY ARTERY NOTINVOLVED,ANEURYSMS
LABORATORY FINDINGS
C-ANCA(>90%) FALSE +VE REPORTED
p-ANCA (>48%) p-ANCA (75%) HEP B ANTIGENEMIA,HAIRY CELL LEUKEMIA
LARGE SIZED VESSELS VASCULITIS SYNDROME
TAKAYASU’S ATERITIS (NON-SPECIFIC AORTO-ARTERITIS)
GIANT CEL ATERITIS (TEMPORAL ARTERITIS)
SPECIFIC FEMALE SEX PREDILICTIONS
MORE COMMON IN ASIA M:F- 1:1.6 (INDIA) 1:9 (JAPAN)AGE: 15-25 YEARS
C/F: GENERAL SYMPTOMS WITH VASCULAR SYMPTOMS HYPERTENSION IS MOST COMMON PRESENTATION.
DISEAESE CLASSIFY ON THE BASIS OF SITE OF INVOLVEMENT OF AORTA AND ITS BRNCHES. MIXED INVOLVEMENT IS THE COMMONEST IN INDIA.
MORE COMMON IN FEMALE
MORE COMMON IN WESTERN COUNTRIESRARE IN INDIA
AGE: MORE THAN 50 YEARS
ASSOCIATED WITH POLYMYALGIA RHEUMATICA.
DISEASE INVOLVES CHARECTERISTICALLY ONE OR MORE BRANCHES OF CAROTID ARTERY.
CLASSIFICATION OF TAKAYASU’S ARTERITIS
SUMMARY• PATIENT SUSPECTED FOR PRIMARY VASCULITIS.
• RULE OUT THE CAUSES WHICH CAN MIMIC VASCULITIS.
• LOOK FOR DEMOGRAPHIC CHARACTERISTICS LIKE AGE,SEX, ETHINICITY,SMOKING STATUS.
• DETERMINE THE SIZE OF VESSELS INVOLVED AND CATEGORIES THE TYPE OF VASCULITIS.
• EXTENT OF ORGAN DAMGE TO BE ASSESSED.
• SPECIFIC LABORATORIES WORK UP REQUIRED FOR CONFIRMATION OF DIAGNOSIS.
• DECIDE THE APPOPRIATE TREATMENT
THANK YOU