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Vasculitis syndrome an approach -and-basic principles of treatment
 

Vasculitis syndrome an approach -and-basic principles of treatment

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Vasculitides are a hetrogenous group of conditions characterized by inflammation and necrosis of blood vessels. ...

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.

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    Vasculitis syndrome an approach -and-basic principles of treatment Vasculitis syndrome an approach -and-basic principles of treatment Presentation Transcript

    • Vasculitis Syndrome An Approach And Basic Principles of Treatment Dr. Sachin Verma MD, FICM, FCCS, ICFC Fellowship 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 LieDOMINENT PRIMARY VASCULITIS PATHOGENESISVESSELSLARGE ARTERIES Temporal arteritis T cell mediated Takayasu’s arteritisMEDIUM ARTERIES Classical PAN Immune complex related Kawasaki’s disease Antibody mediated hypersensitivitySMALL AND MEDIUM Wegener’s granulomatosisSIZE ARTERIES CSS Antibody mediated MSA (ANCA)SMALL VESSEL VASCULITIS HSP(Leukocytoclastic) EMC Immune complex mediated CUTANEOUS LCV
    • 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, Wegeners granulomatosis, Hypersensitivity vasculitis, Henoch-Schönlein purpura, Giant cell arteritis, Takayasus arteritis, Granulomatous angitis of CNS, Bergers 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. PERCENTAGEAortoarteritis 215 20.20Giant cell arteritis 35 3.36Polyarteritis nodosa (PAN) 95 8.83Cutaneous PAN 13 1.22Wegener’s granulomatosis 147 13.83Microscopic polyangiitis 42 3.94Churg Straus syndrome 19 1.78Henoch Schonlein purpura 232 21.80Small vessel vasculitis 61 5.73Behcet’s disease 145 13.62Kawasaki disease 05 0.46Undiagnosed 50 4.69Others** 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 : A GUIDE TO THE TYPE OF VESSELS INVOLVED CLINICAL MANIFESTATIONS:LARGE VESSELS MEDIUM VESSELS SMALL VESSELSLimb claudication Red and blue Panniculitis PurpuraAsymmetric blood pressure Ulcer Vesicobullous lesionsAbsence of pulses Livedo reticularis. UrticariaBruits Digital gangrene GlomerulonephritisAortic dilatation Mononeuritis multiplex Alveolar haemorrhageRenovascular 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, PainEye ,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 ProteinuriaSerum proteins: Hypergammaglobulinemia (mostly IgG type, IgA in HSP, WG) Complements levels usually decreasedAntineutrophil 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 SIZEDVASCULITIS SYNDROMES
    • WEGENNER’S CHURG SRAUSS MICROSCOPIC GRANULOMATOS SYNDROME POLYANGITIS IS PAN 1.2:1 OVER ALL OVER ALL 1:1 NO MUCH DIFF. UNKNOWN UNKNOWN M:F FROM WESTRN 1:14(INDIA) DATA 4.5:1(INDIA)TYPE OF VESSELS SMALL ARTERIES SMALL AND SMALL VESSELS SMALL ANDINVOLVED AND VEINS MEDIUM SIZED (ARTERIES, MEDIUM SIZED VESSELS CAPILLARIES, ARTERIES ONLY VENULES)SPECIFIC TRIAD: UPPER EXTRA PULMONARY PULMONARYFEATURE AND LOWER AIR VASCULAR CAPILLARIES ARTERY NOT WAYS WITH GRANULOMA INVOLVED, INVOLVED, KIDNEY LESIONS GNs +NT ANEURYSMSLABORATORY C-ANCA(>90%) p-ANCA (>48%) p-ANCA (75%) HEP BFINDINGS FALSE +VE ANTIGENEMIA, REPORTED HAIRY CELL LEUKEMIA
    • LARGE SIZED VESSELSVASCULITIS SYNDROME
    •  TAKAYASU’S ATERITIS  GIANT CEL ATERITIS (NON-SPECIFIC AORTO-ARTERITIS) (TEMPORAL ARTERITIS) SPECIFIC FEMALE SEX MORE COMMON IN FEMALEPREDILICTIONS MORE COMMON IN WESTERNMORE COMMON IN ASIA COUNTRIES M:F- 1:1.6 (INDIA) RARE IN INDIA 1:9 (JAPAN)AGE: 15-25 YEARS AGE: MORE THAN 50 YEARSC/F: GENERAL SYMPTOMS WITHVASCULAR SYMPTOMS HYPERTENSION IS MOST COMMON ASSOCIATED WITH POLYMYALGIAPRESENTATION. RHEUMATICA.DISEAESE CLASSIFY ON THE BASIS OFSITE OF INVOLVEMENT OF AORTA AND DISEASE INVOLVESITS BRNCHES. CHARECTERISTICALLY ONE OR MORE BRANCHES OF CAROTID ARTERY.MIXED INVOLVEMENT IS THE
    • 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