VASCULITIS final

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Slide 1: 

Vasculitis-Approach to Diagnosis Chairperson Presented by Guide Prof.Dr.A.G.Shingwekar Dr.Ankit Dr.Y.S.Verma HOD RMO Assistant Prof.

What is Vasculitis? : 

What is Vasculitis? A general term for a group of diseases that involve inflammation of the blood vessel There are 20 different types of Vasculitis All blood vessels can be affected from the largest (Aorta) to smallest blood vessels in the skin (capillaries) Relatively common 1:600 though sub categories can be rare. Severity can range from benign (Behcet’s, Kawasaki) to fatal (Wegener’s, Polyarteritis Nodosa) 16 September 2010 2

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16 September 2010 3

Symptoms …. : 

Symptoms …. Vasculitis is a “hurting disease”:: Pain from nerve infarction Pain from sore joints Pain from ulcers Pain from insufficient blood flow to the GI tract Vasculitis may involve virtually every organ system in the body Often have fever, weight loss, fatigue, a rapid pulse, and diffuse aches and pains that are difficult to pinpoint 16 September 2010 4

What Causes the Symptoms??? : 

What Causes the Symptoms??? Inflammation: Of the blood vessel itself Inflammation: Of other tissues in the body Ischemia: From lack of blood flow through the swollen blood vessel Damage: From longstanding lack of blood flow or inflammation 16 September 2010 5

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16 September 2010 6 Organ Systems Affected…. Skin Joints Heart Brain Kidneys GI Tract Sinuses, nose, & Ears Nerves Lungs Eyes

Skin: A Variety of Rashes…. : 

Skin: A Variety of Rashes…. Palpable Purpura: Most common rash Begin as non-palpable petechiae or purpura which coalesce with time Vary in size and usually found on the lower limbs (dependent) Become palpable with time Do not blanche. 16 September 2010 7

Other Rashes : 

Other Rashes Urticaria (Hives) Skin Ulcers Skin Blisters Skin Nodules Erythematous plaques 16 September 2010 8

Joints : 

Joints 16 September 2010 9 Arthritis: Full blown swelling of the joints Arthralgia: Aching and pain in the joints with little swelling Eg. PAN,WG,Churg- Strauss Vasculitis

Nerves : 

Nerves Can affect the blood supply to nerves and result in : Mononeuritis: Damage to a single nerve Mononeuritis-Multiplex: Damage to many single nerves (i.e. Ulnar, Sural, peroneal etc) Eg. PAN,WG,Churg Strauss Cryoglobulinemia 16 September 2010 10

Lungs : 

Lungs Cough Shortness of breath WG Hemoptysis MPA Infiltrates in the lungs (inflammatory) Churg-Strauss Cavities in the lungs from MPA necrosis or inflammation 16 September 2010 11 Inflitrates in the lungs which represent bleeding from damaged capillaries NOT infection

Heart : 

Heart Inflammation of coronary blood vessels Ischemia/Infarction Inflammation of other tissues Pericarditis Myocarditis Endocarditis 16 September 2010 12

Normal CXR : 

Normal CXR 16 September 2010 13

Pericarditis with Effusion : 

Pericarditis with Effusion 16 September 2010 14

Kidneys : 

Kidneys 16 September 2010 15 Inflammation of the kidney glomeruli may lead to: Protein in the urine Blood in the urine Red Blood Cell Casts in the urine Renal failure with rising creatinine Can be very severe and rapid Eg. HSP,WG,MPA,PAN,C-S

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16 September 2010 16

Gastrointestinal Tract : 

Gastrointestinal Tract Inflammation of the blood vessels supplying the GI Tract leads to ischemia and: Abdominal pain Bloody diarrhea Sometimes the bowel perforates Eg. HSP,PAN,Churg- Strauss vasculitis 16 September 2010 17

Sinuses, Nose, & Ears : 

Sinuses, Nose, & Ears Inflammation of the organs or the blood vessels supplying the organs. Chronic sinus “infections” with nose bleeds. Inflammation of the nasal septum – saddle nose deformity. Inflammation of the ears. Hearing loss. 16 September 2010 18

Eyes : 

Eyes Blood vessels going to the eyes Sudden loss of vision. Blood vessels within the eye Conjuctivitis (pink-eye). Thinning of the sclera (white part of the eye). Inflammation in other chambers (iritis). Retinal vasculitis. 16 September 2010 19

Brain : 

Brain Headaches Strokes Changes in mental status Difficulty with coordination Eg.GCA,SLE associated 16 September 2010 20

Classification of Vasculitis : 

Classification of Vasculitis Vasculitis may be classified by: The size and type of vessel involvement The histopathologic features (leukocytoclastic, granulomatous vasculitis, etc.) The pattern of clinical features 16 September 2010 21

CLASSIFICATION TREE : 

CLASSIFICATION TREE 16 September 2010 22 Vasculitis Large Blood Vessel Takayasu Arteritis Giant cell arteritis Medium Blood Vessel Polyarteritis Nodosa Kawasaki’s Disease Rheumatoid vasculitis Small Blood Vessel ANCA Associated Wegener’s Granulomatosis Churg-Strauss Vasculitis Microscopic Polyangiitis Non-ANCA Associated Immune Complex Hypersensitivity Vasculitis Cryoglobulinemic Vasculitis CTD related Vasculitis Henoch Schonlein Purpura Behcet’s Disease Miscellaneous Paraneoplastic Vasculitis Inflammatory Bowel Disease

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16 September 2010 23

Henoch Schonlein Purpura… : 

Henoch Schonlein Purpura… Small vessel vasculitis. Diagnostic criteria: Palpable purpura + any one of : Diffuse abdominal pain. Renal involvement. Arthritis/arthralgia Predominant IgA deposition 16 September 2010 24 vasculitis-like” hemorrhage, small ulcers and edema of the duodenum

Kawasaki disease… : 

Kawasaki disease… All sizes of blood vessels involved but predominantly affects medium sized arteries. Diagnostic criteria: Fever for at least 5 days + Any 4 of the following: Bilateral non purulent bulbar conjunctivitis. Changes in extremities Acute:Erythema of palms,soles;hand & feet edema Subacute: Periungual peeling of fingers & toes (2 & 3 week) Changes in lips & oral cavity. Polymorphous rash,primarily truncal Cervical lymphadenopathy(>1.5 cm) usually unilateral 16 September 2010 25

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26 16 September 2010

Polyarteritis nodosa…. : 

Polyarteritis nodosa…. Middle-aged and predominantly have medium-sized artery vasculitis Associated with -- "CLASH"--cryoglobulinemia, leukemia, arthritis, Sjögren's syndrome and hepatitis. Diagnostic criteria: Biopsy showing necrotizing vasculitis or angiographic abnormalities. + Atleast two of the following: Cutaneous involvement. Myalgia Systemic HT Renal involvement Mononeuropathy/Polyneuropathy Testicular tenderness Other system involvement(GIT/CVS/CNS/Pulmonary) 16 September 2010 27

Takayasu arteritis… : 

Takayasu arteritis… Primarily large vessel vasculitis,aorta & its branches. Diagnostic criteria: Angiographic evidence of involvement of aorta/major branches + Any one of the following: Absence peripheral pulses/extremities of claudication Systolic difference > 10 mm Hg in both arms Systemic HT Bruit over aorta or carotid arteries 16 September 2010 28

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16 September 2010 29

Approach to Vasculitis : 

Approach to Vasculitis Attempt to exclude other processes, particularly infection, thrombosis and neoplasia. Consider the patient's age, sex. Determine the type and extent of organ system involvement. Determine a “pattern of clincal features” which may be helpful in classification. 16 September 2010 30

Demographic associations….. : 

Demographic associations….. 16 September 2010 31

When to suspect vasculitis??? : 

When to suspect vasculitis??? 16 September 2010 32

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Laboratory investigations… : 

Laboratory investigations… 16 September 2010 35

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Slide 37: 

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Diagnosis of Vasculitis : 

Diagnosis of Vasculitis Places to Biopsy Skin & Underlying Nodules Kidneys Nerves (Sural nerve; ‘sensory nerve’) Temporal Artery Lung Biopsy Brain Biopsy 16 September 2010 38 Multi-Nucleated Giant Cell

Slide 39: 

16 September 2010 39

Conditions that mimic vasculitis… : 

Conditions that mimic vasculitis… 16 September 2010 40

Pit falls in the diagnosis… : 

Pit falls in the diagnosis… Angiographic appearance of diseases such as fibromuscular dysplasia, atherosclerosis and drug-induced vasospasm can be similar. Small-vessel involvement in the skin or visceral organs can also be the result of immune complex deposition. Skin biopsy showing leukocytoclastic vasculits does not prove true systemic or visceral involvement nor does it prove or disprove involvement of larger vessels. Vasculitic lesions tend to be focal and segmental. 16 September 2010 41

Slide 42: 

A renal biopsy only rarely reveals vasculitis in the renal vessels themselves. In addition, renal biopsy findings usually cannot differentiate the type of vasculitis. None of the pathologic processes that occur in vasculitis are diagnostic of a specific vasculitic syndrome. 16 September 2010 42 THINK FOR A SECOND

Take home message… : 

Take home message… Vasculitis is an inflammation of blood vessels,any organ system can be involved. After infections it is the second most common cause of multi system involvement in children. All the features of different types of vasculitis need not to be present in a single patient-- Undifferentiated Systemic Vasculitis 16 September 2010 43

Vasculitis is ... : 

Vasculitis is ... Frustrating as signs and symptoms may be non-specific Vasculitis can be horrible Worrisome as it can result in death 16 September 2010 44

References… : 

References… Nelson textbook of Pediatrics,18th Edition Ghai Textbook of Pediatrics www.wikipedia.org An Approach to Diagnosis and Initial Management of Systemic Vasculitis,DOUGLAS W. ROANE and DONALD R. GRIGER, Other References: American Family PhysicianAn Approach to Diagnosis and Initial Management of Systemic Vasculitishttp://www.aafp.org/afp/991001ap/1421.htmlANCA­Associated Small-Vessel Vasculitis http://www.aafp.org/afp/20020415/1615.html Johns Hopkins Vasculitis Centerhttp://vasculitis.med.jhu.edu/index.html 16 September 2010 45

Thanks : 

Thanks 16 September 2010 46