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VASCULITIS
• MOST COMMON VASCULITIS IN CHILDREN IS HSP
• MOST COMMON VASCULITIS CAUSING DEATH IN CHILDREN IS
KAWASAKI
• MOST COMMON VASCULITIS IN ADULT IS GIANT CELL ARTERITIS
GIANT CELL ARTERITIS
• FOCAL GRANULOMATOUS INFLAMMATION OF CRANIAL VESSELS
MAINLY SUPERFICIAL TEMPORAL ARTERIES
• ALSO INVOLE VERTEBRAL & OPHTHALMIC ARTERIES
• INVOLVEMENT OF OPHTHALMIC ARTERY  BLINDNESS
ASSOCIATED WITH
POLYMYALGIA
RHEUMATICA
RAISED ESR
CF
• JAW CLAUDICATION  MOST SPECIFIC SYMPTOM
• TEMPORAL HEADACHE  MOST COMMON SYMPTOM
SEGMENTAL NODULAR
THICKENING
DIAGNOSIS
• ELEVATED ESR
• TEMPORAL ARTERY BIOPSY
FRGAMENTATION &
MULTIPLICATION OF
INTERNAL ELASTIC LAMINA
MULTINUCLEATE GIANT
CELLS
NARROW LUMEN
TREATED WITH STEROIDS
TAKAYASU ARTERITIS
TAKAYASU ARTERITIS
• PULSELESS DISEASE
• MARKED WEAKENING OF PULSE OF UPPER LIMB ARTERIES
• UNEQUAL BP
• AORTIC ARCH SYNDROME
• GRANULOMATOUS VASCULITIS OF AORTIC ARCH OR ITS BRANCHES
YOUNG INDIVIDUALS
<50 YEARS
• MOST COMMONLY INVOLVES SUBCLAVIAN VESSELS
• LEAST COMMONLY INVOLVED  CORONARY ARTERIES
INVESTIGATIONS
• TRANSESOPHAGEAL ECHO
• ANGIOGRAPHY
Rx OF TAKAYASU
• BALLOON DILATION & STENTING
MEDIUM VESSEL VASCULITIS
POLYSRTERITIS NODOSA
• NECROSTISING VASCULITIS
OF MEDIUM SIZED
MUSCULAR ARTERIES
• CAUSES FIBRINOID
NECROSIS
• MOST COMMONLY AFFECT
KIDNEY & PULMONARY
CAPILLARIES ARE SPARED
• ANCA IS NOT POSITIVE
• ASSOCIATED WITH
HEPATITIS B
CF
• MOST COMMON
VASCULITIS A/W
MONONEURITIS
MULTIPLEX
• MOST COMMON CAUSE OF
MONONEURITIS IS DM (BUT AMONG
VASCULITIS MOST COMMON CAUSE IS
• PAN IS ASSOCIATED WITH
LIVIDO RETICULARIS
SEGMENTAL
TRANSMURAL
NECROTISING
INFLAMMATION OF
ARTERIES ELASTIC
LAMINA
FRAGMENTATION &
FIBRINOID NECROSIS
MOST COMMON CAUSE OF
DEATH IS RENAL FAILURE
• ALL STAGES OF ACTIVITY CO EXIST IN DIFFERENT VESSELS
• RX
• STEROIDS
KAWASAKI DISEASE
• MUCOCUTANEOUS LN SYNDROME
• INFECT CHILDREN 3MONTHS TO 5 YEARS
• ANTIENDOTHELIAL Ab
RED DRY CRACKED LIPS
NON SUPPURATIVE
CONJUNCTIVITIS
STRAWBERRY
TONGUE
PERIUNGAL PEELING OF SKIN
Rx
• IVIG
COMPLICATIONS
CORONARY DUCT
ECTASIA & CORONARY
ANEURYSM
MAIN CAUSE OF DEATH IS
MYOCARDIAL INFARCTION
ECHOCARDIOGRAPHY IS DONE TO
DETECT CORONARY ARTERY
ANEURYSM
SMALL VESSEL VASCULITIS
MICROSCOPIC POLYANGITIS
• HYPERSENSITIVITY VASCULITIS OR LEUKOCYTOCLASTIC VASCULITIS
• POSITIVE p ANCA
MICROSCOPIC POLYANGITIS
• NO GRANULOMA
• SEGMENTAL FIBRINOID NECROSIS OF MEDIA
• POST CAPILLARY VENULES SHOW FRAGMENTED NEUTROPHILIC
NUCLEI
• NECROTISING GN
NEUTROPHIL NUCLEAR REMNANTS
AROUND SMALL VESSELS
FIBRINOID NECROSIS OF TUNICA MEDIA
NECROTISING GLOMERULONEPHRITIS
• MPA IS NOT HAVING
• ANY GRANULOMA OR NODULES & CAVITIES IN LUNGS IN MPA
• UNLIKE IN WEGNWERS
WEGENERS GRANULOMATOSIS
• NECROTISING SMALL VESSEL VASCULITIS
NECROTISING OR
GRANULOMATOUS VASCULITIS
OF UPPER & LOWER AIRWAY
NECROTISING
GRANULOMATOUS OF LUNGS
FOCAL NECROTISING &
CRESCENTIC GN
LIMITED FORMS OF DISEASE
WILL AFFECT RESPIRATORY
TRACT
MULTIPLE CAVITARY
LESIONS
FOCAL NECROTISING GN
CF
• PERSISTENT PNEUMONITIS WITH B/L NODULAR & CAVITARY
INFILTRATES
• C/C SINUSITIS
• MUCOSAL ULCERATION OF NASOPHARYNX
• RENAL LESIONS  HEMATURIA
• GRANULOMA WITH
GEOGRAPHIC NECROSIS
• GRANULOMA WILL
FORM NODULES &
CAVITIES
STAIN +VE FOR c ANCA & p ANCA
SADDLE NOSE DEFORMITY IN WEGENERS
GRANULOMATOSIS
Rx
• STEROIDS
• CYCLOPHOSPHAMIDE
• TNF α AGONIST
CHURG STRAUSS SYNDROME
• ALLERGIC GRANULOMATOSIS & ANGITIS
• SMALL NECROTISING VASCULITIS WITH
• ASTHMA
• ALLERGIC RHINITIS
• PERIPHERAL EOSINOPHILIA
• EXTRAVASCULAR NECROTISING GRANULOMA
CHURG STRAUSS SYNDROME EOSINOPHILIC GRANULOMA
• EOSINOPHILIC
INFILTRATE ARE
ASSOCIATED WITH
CARDIOMYOPATHY &
CARDIAC
INVOLVEMENT 
MOST COMMON CAUSE
OF MORTALITY
EOSINOPHILIC INFILTRATE IN
MYOCARDIUM
HENOCH SCHONLEIN PURPURA
• MOST COMMON VASCULITIS IN CHILDREN
• MOST COMMONLY SEEN IN EXTENSOR SURFACE  EXTENSOR
PURPURA
• IgA ELEVATED ABNORMALLY
MOST COMMONLY SEEN IN
EXTENSOR SURFACE
PALAPABLE PURPURA D/T VASCULITIS
(NOT D/T THROMBOCYTOPENIA)
DIAGNOSIS
• ELEVATED IgA
• IOC  SKIN BIOPSY
• PLATELET IS NORMAL
THROMANGITIS OBLITERANS
• BUERGERS DISEASE
• SMOKERS
• YOUNG AGE < 35 YEARS
• INVOLVES SMALL ARTERIES WITH SECONDARY EXTENSION IN TO VEIN
AND NERVES
HLA B12 IS
PROTECTIVE
AGAINST
TAO
HLA A9 & HLA B5
INCREASED RISK
PATHOLOGY IN TAO
NEUTROPHILIC
MICROABSCESS FORMATION
SURROUNDED BY
GRANULOMATOUS
INFLAMMATION
CF
• SEGMENTAL THROMBOSING VASCULITIS EXTENDING IN TO VEINS &
NERVE
• PAIN EVEN AT REST D/T NEURAL INVOVEMENT
• INTERMITTENT CLAUDICATION
PERINUCLEAR ANCA (p ANCA)
MYELOPEROXIDASE
• CHURG STRAUSS
SYNDROME
• GOOD PASTURE SYNDRME
• MICROSCOPIC POLYANGITIS
• IDIOPATHIC CRESCENTIC GN
CYTOPLASMIC ANCA ( c
ANCA)
PROTEINASE 3
• WEGENER
GRANULOMATOSIS
(GRANULOMATOSIS WITH
POLYANGITIS)
WEGENERS GRANULOMATOSIS
BEHCET DISEASE
BEHCET DISEASE
• ORO OCULO GENITAL SYNDROME
• SMALL VESSEL NEUTROPHILIC VASCULITIS
BEHCET DISEASE
• HLA B5 & HLA B51 ASSOCIATION
RECURRENT ORAL ULCER
RECURRENT GENITAL ULCER
POSITIVE PATHERGY TEST
STERILE PUSTULE AFTER 24 – 48 HOURS 
POSITIVE PATHERGY TEST

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