VASCULITIC SYNDROMES. Howard L. Feinberg, D.O., F.A.C.O.I., F.A.C.R. OPSC 2018

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1 VASCULITIC SYNDROMES Howard L. Feinberg, D.O., F.A.C.O.I., F.A.C.R. OPSC 2018

2 2012 REVISED CHAPEL HILL CONSENSUS CONFERENCE Large vessel Takayasu arteritis Giant cell arteritis Medium Vessel Polyarteritis nodosa Kawasaki disease Variable Vessel Bechet s disease Cogan s syndrome Small vessel ANCA Immune complex Anti-GBM Cryoglobulinemia HSP (IgA vasculitis) Anti-C1q vasculitis (hypocomplementemic urticarial vasculitis)

3 PRIMARY VASCULITIC DISEASES Takayasu s Arteritis Giant Cell Arterits Isolated CNS Vasculitis Polyarteritis nodosa (Eosinophilic Granulomatosis with Polyangiitis (Churgg- Strauss vasculitis) Microscopic polyangiitis (MPA) Granulomatosis with Polyangiitis (Wegeners Granulomatosis) IgA Hypersensitivity Vasculitis (Henoch- Schonlein purpura) Bechet s Beurgers Cogan s Kawasaki disease

4 SECONDARY VASCULITIC DISEASES Infection related vasculitis Secondary to connective tissue disease Vasculitis secondary to mixed cryoglobulinemia Malignancy associated vasculitis Hypocomplementemic urticarial vascultits Post-organ transplant vasculitis Pseudovasculitis syndromes antiphospholipid syndrome atrial myxoma endocarditis Sneddon s syndrome

5 COMMON CLINICAL SYMPTOMS Constitutional Features Rashes Muskuloskeletal Arthritis myositis weakness Arthralgias Neuropathy Mononeuritis multiplex Sensory peripheral neuropathy Respiratory Alveolitis - Pulmonary hemorrhage Nodules Infiltrates Asthma Sinusitis

6 COMMON CLINICAL SYMPTOMS Renal Hypertension Proteinuria Necrotizing GN Gastrointestinal Diarrhea Nausea Abdominal pain Hemorrhage Elevated LFT s Claudication Jaw Extremities Central Nervous System Headache Stroke Seizures Altered mentation Visual changes Cranial nerve abnormalities

7 POLYARTERITIS NODOSA Disease of small and medium sized blood vessel Peak age male : female 2:1 May occur as a primary or secondary disease

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9 CLASSIFICATION CRITERIA Weight loss > 4kg Livido reticularis Testicular pain Myalgias, weakness, or leg pain Mononeuropoathy or polyneuropathy New onset hypertension Elevated BUN, creatnine Hepatitis B surface Ag Positive arteriogram (aneurysms or occlusions) Biopsy showing granulomas

10 ASSOCIATED DISEASES Hepatitis B or C Acute otitis media Streptococcal infections SLE RA Sjogren s syndrome Dermatomyositis Scleroderma Relapsing polychondritis Cryoglobulinemia Inflammatory bowel disease Post vascular surgery Allergy hyposensitization therapy

11 TREATMENT Corticosteroids Cyclophosphamide

12 PROGNOSIS One year survival 85% Poor prognosis with: HBsAg positive 70% cardiac disease 70% current infection 10% renal failure 40%

13 MICROSCOPIC POLYANGIITIS Similar symptoms to PAN P-ANCA (MPO) No granulomas Alveolar hemmorhage Segmental necrotizing GN

14 Renal - Limited P-ANCA (MPO) Pauci-immune GN Limited only to the kidney

15 Eosinophillic Granulomatosis with Polyangiitis (CHURGG-STRAUSS VASCULITIS) Asthma Eosinophilia > 10% on differential Mononeuropathy and/or Mononeuritis multiplex Nonfixed pulmonary infiltrates Abnormal paranasal sinuses Biopsy with a blood vessel showing extravascular eosinophils P-ANCA (MPO)

16 Eosinophillic Granulomatosis with Polyangiitis Pulmonary infiltrates nodules hilar adenopathy pleural effusions interstitial disease asthma Skin palpable purpura nodules Renal - rare Laboratory Hypocomplementemia -rare increased ESR eosinophils

17 Eosinophillic Granulomatosis with Polyangiitis Treatment Mepolizumab (Nucala) FDA approved therapy Prednisone oral I.V. Cyclophosphamide Azothiaprim

18 Eosinophillic Granulomatosis with Polyangiitis Survival 90% - 1 year 76% - 3 year 62% - 5 year 0% without treatment Long term sequelae hypertension peripheral neuropathy renal insufficiency

19 Granulomatosis with Polyangiitis (WEGENER S GRANULOMATOSIS) Necrotizing granulomatous vasculitis of the upper and lower respiratory tract Systemic vasculitis small- medium size vessels Focal Necrotizing glomerulonephrosis canca (anti pr3)

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21 Granulomatosis with Polyangiitis Prognosis without cyclophosphamide average survival 5 months 85% mortality at 1 year With cyclophosphamide 75% remission 91% improvement 50% relapse in 3 years Prednisone - prolongs survival from month average

22 Granulomatosis with Polyangiitis Clinical sinusitis glomerulonephriis pulmonary Treatment Cyclophosphamide symptomatic methotrexate Rituximab

23 IgA Hypersensitivity Vasculitis (HENOCH-SCHONLEIN PURPURA) Small vessel vasculitis 13.5/100,000 in pediatric population male > female peak age 2-11

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25 IgA Hypersensitivity Vasculitis Nonthrombocytopenic purpura Arthritis Abdominal pain G.I. Hemorrhage Glomerulonephritis major source of morbidity and mortality occurs in 20%

26 IgA Hypersensitivity Vasculitis Treatment oral prednisone I.V. prednisone Cytotoxic - rarely required No treatment

27 TAKAYASU S DISEASE Large vessel vasculitis aorta aortic branches pulseless disease Predominantly affects women under age 40 Claudication Decreased Brachial pulse BP difference >10 between arms Bruit over Aorta or subclavian arteries Abnormal angiogram

28 TAKAYASU S DISEASE Clinical dizziness headache malaise fever weight loss visual changes Physical findings bruits loss of pulse claudication retinal aneurysms, venous dilation, beading Cardiac CHF aortic dilitation aortic valve disease Pulmonary vascular disease

29 TAKAYASU S DISEASE Treatment Prednisone oral 60mg/day I.V. - works in 50% of non-responders Cyclophosphamide Methotrexate Cyclosporin A

30 TAKAYASU S DISEASE Prognosis 25% at two years without treatment 95% at two years with treatment Long term survival varies in reports Major causes of death CHF Stroke MI renovascular hypertension unresponsive to medical therapy

31 TEMPORAL ARTERITIS Incidence 24/100,000 Rare under age 50 Predominantly Caucasian female > male Associated with Polymyalgia Rheumatica

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33 TEMPORAL ARTERITIS Symptoms Headache > 50% Abnormal temporal artery > 50% Jaw claudication Scalp tenderness Weight loss Fever Depression Facial neuralgia Blindness Vertigo Stroke Angina Claudication of tongue Claudication of extremities

34 TEMPORAL ARTERITIS Treatment Prednisone Treatment required for 1-3 years Prognosis Good with early diagnosis and treatment

35 URTICARIAL VASCULITIS Recurrent or persistent urticarial lesions due to vasculitis Diagnosed based on biopsy findings of vasculitis in the face of urticaria Benign chronic course Systemic symptoms fever abdominal pain renal disease arthralgias obstructive pulmonary disease renal

36 KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME) Primarily affects infants and young children Mainly affects coronary arteries Fever > 5 days Bilateral conjunctival injection Strawberry tongue Diffuse erythema of oropharynx Palmar erythema erythema of the soles of the feet Desquamation Cervical lymphadenopathy

37 KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME) Myocarditis occurs in 50% MI occurs in 1% Coronary artery aneurysms 25% Leading cause of acquired heart disease in the pediatric population Treatment Prednisone I.V. Ig Aspirin Infliximab Echocardiogram Baseline 2 week 6-8 week 6 month?

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39 COGAN S SYNDROME Interstitial keratitis Sensorineural hearing loss (deafness in 50%) Tinnitus Vertigo Photophobia Eye Pain Treatment Topical steroids - eye Systemic steroids Cytotoxic - no benefit

40 DEGO S SYNDROME Erythematous papules with porcelain white centers and peripheral telangiectatic ring Genetic small to medium vessel vasoocclusive disease Thrombosis (?due to clotting abnormality C5) Central retinal artery necrosis Perforation of intestine causing death - 50% Stroke Some patients have a benign course for unknown reason Treatmenteculizumab

41 THROMBOANGIITIS OBLITERANS (BUERGER S DISEASE) A disease in which an acute inflammatory lesion and occlusive thrombosis of the arteries and veins are the characteristic lesions Begins distal and moves proximal Rest pain - 81% Ischemic ulcers 76% Raynaud s Neuropathy Digital infarcts Treatment - abstinence from tobacco

42 MIXED CRYOGLOBULINEMIA Clinical Triad Purpura Arthralgias Weakness Most common in middle aged women Associated with Hepatitis C Laboratory ESR, anemia, hypocomplementemia Treatment 50% have a benign course and require no treatment Indications for treatment - renal or neurologic dysfunction Prednisone Cytotoxic Plasmaphereisis Interferon alpha

43 HOWARD L. FEINBERG, D.O.,F.A.C.O.I., F.A.C.R.