JMJ Connective Tissue Disorders K. Kavindya M. Fernando

Vasculitides

Large vessel vascilitis Medium vessel vasculitis Small vessel vasculitis

Giant cell artritis &

polymyalgia

rheumatic

Takayasu’s artritis

Polyartritis nodosa

Kawasaki disease

Wegener’s

granulomatosis*

Churg-Strauss

Syndrome*

Microscopic

polyangitis*

Henoch-Schonlein

purpura

Essential

cryoglobulenemic

vasculitis

* Vascilitides most commonly associated with ANCA

Mnemonic

TT was given to PK coz he didn’t Wear MCR to the BE(a)CH

Large vessel – Temporal / Takayasu

Medium Vessel – Polyarthritis /Kawasaki

Small vessel- ANCA (+) – Wegeners/Microscopic/ chaurg

Small vessel ANCA (-) – Behcet’s / cryoglobulenemic

Large vessel vasculitis

Giant cell artritis Takayasu’s Artritis

Other names Temporal artritis

Occur association with

PMR

Pulseless disease

Aortic arch Xn

Prevelance Increase with age

Usually over 50

Most common vasculitis

Affects Cerebral vessels

Only the extracranial

vessels

Aorta and its main branches

Pathology Focal granulomatous

inflammation of the

intima & media

Braking up of the internal

elastic lamina

Symptoms Severe Headache

Temporal artery tender,

erythematous

Scalp tender

Jaw claudication

Lingual claudication

Pain and tenderness over

the affected vessles

Absent peripheral pulse

Hypertension

JMJ Connective Tissue Disorders K. Kavindya M. Fernando

Investigations ESR > 50mm/hr

Conformation temporal

artery biopsy

Skip lesions

Liver biochemistry – due

to PMR

Treatment Prednisolone

Normalization of ESR and

symptoms

Up to 2 years

Corticosteroids for

constitutional symptoms

Surgical Tx to bypass

affected artery

Can develop heart failure

and stroke

Can survive for 5 years

Medium Vessel vasculitis

Polyartritis Nodosa Kawasaki disease

Other names AKA mucocutaneous lymph

node syndrome

Prevalence Male>female

50-60 years

Peadiatric age group

Hepatitis B antigen in 30%

Aneurysms

Affected tissues GI tract

Hepatobiliary system

Kidney

Pancreas

Skeletal muscle

Complaints /

clinical features

Non-specific multisystem

Malaise, weight loss,

anorexia, fever

Fever,

red dry lips,

erythema oral mucosa,

polymorphous truncal rash,

desquamation fingers and

toes,

cervical lymphadenopathy

Signs Progressive arthritis

Myopathy

Neuropathy

Hepatic & renal failure

GI bleeding

JMJ Connective Tissue Disorders K. Kavindya M. Fernando