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39 Cards in this Set

  • Front
  • Back

3 mechanisms of Vascultits

1. pathogenic immune complex formation/ deposition


2. production of ANCA


3. Pathogenic T lymph responses & granuloma formation

What diseases cause pathogenic immune complex formation/deposition vasculitis?

Henoch-Schonlein purpura


Vasc assoc w/ CVD


Serum sickness & cutaneous vasc syndromes


Hep C (mixed cryoglobinemia)


Hep B (Polyarteritis nodosa)

What diseases cause production of ANCA leading to vasculitis?

Wegner's Granulomatosis


Churg Strauss syndrome


microscopic polyangiitis

what diseases cause pathogenic T lymph responses & granuloma formation leading to vasculitis?

Giant Cell arteritis


Takayasu's arteritis


Wegners Granulomatosis


Churg Strauss syndrome

Vasculitis in inflammation of blood vessels that leads to ......

narrowing of the lumen



(due to thickening of the walls)



(may eventually lead to blockage, aneurysm, or rupture)

What pathology is this?
What do the purple dots represent?

What pathology is this?


What do the purple dots represent?

Panarteritis (Vasculitis)



purple dots= inflammatory cells w/i the vessel

Constitutional symptoms of Vasculitis:

fever


weight loss


malaise


arthralgias/arthritis

Symptoms associated w/ large vessel vasculitis

Limb claudication (cramping)


Asymmetric BP


Absence of pulses


Bruits


Aortic dilation

Large & medium vessel vasculitis

Takayasu's Arteritis (pulseless)


Giant cell arteritis (Temporal)

Symptoms associated w/ medium vessel vasculitis

cutaneous nodules


ulcers


livedo reticularis


digitial gangrene


mononeuritis multiplex


microaneurysma


medium & small vessel vasculitis

kawasaki disease


Polyarteritis nodosa (PAN)


Wegners granulomatosis


Churg-Strauss syndrome (allergic granuloma & angitis)


Symptoms associated w/ small vessel vasculitis

purpura


vesiculobullous


lesions


urticaria


glomulonephritis


alveolar hemorrhage


cutaneous extra-vascular necrotizing granulomas


splinter hemorrhages


uveitis


episcleritis


scleritis

Small vessel vasculitis

necrotizing glomerulonephritis


microscopic polyangitis


Henoch-Schonlein purpa


Hypersensitivity Vasulitis


Cryoglobinemia

What lab tests indicate vasculitis?

anemia


high LFT (liver enzymes inc)


hematuria


high ESR (segmentation rate = inflammation)

What is wrong w this finger?

What is wrong w this finger?

purpuric lesion (or infarct)



= small vessel vasculitis

What is wrong in this picture?

What is wrong in this picture?

retinal vessels leaking exudates



=retinal vasculitis

What is wrong in this pic?

What is wrong in this pic?

scleritis & episcleritis

What is wrong in this image?

What is wrong in this image?

cavitary pulmonary lesion w/ inflammation

What pathology?


What is the sample of?


What is the yellow portion (red bracket)?

What pathology?


What is the sample of?


What is the yellow portion (red bracket)?

Cryoglobulinemia (causing purpura on skin)



-serum sample w/ yellow cryocrit



(must take blood & keep at body temp until serum extracted, then refridgerate to seperate cryocrit (immune complex deposits))

Palpable purupra occurs in what conditions?

Palpable purupra occurs in what conditions?

-serum sickness


-Henoch-schoenlein purpura


-essential mixed cryoglobinemia

Palpable purpura is clinically classified as ___________________



What causes the painful purple sores?

Small vessel vasculitis/ Leukocytoclastic vasculitis (LVC)



-skin w/ PMNs & mononuclears cells leading to fibrinoid necrosis & wbc fragments (= purpura)

Which disease?


-typical in children


-seasonal (spring)


-immune-complex depos. of IgA


-Arthritis


-Nephropathy


-GI (bleeding, abdominal pain)


-Purpura


-small vessel vasculitis

Henoch-Schoenlein Purpura

What disease?


-mixed immun-complex depos. of IgG & IgA


(cold precip. complexes)


-often have a + rheumatoid factor (w/o RF)


-polyarthralgia


-Nephritis


-Peripheral neuropathy


-Assoc. w. Hep C


-Lower extremity purpura


-small vessel vasculitis


Essential Mixed Cryoglobinemia


 


(essential if in absense of another disease)

Essential Mixed Cryoglobinemia



(essential if in absense of another disease)

In addition to purpura, what are the other clinical symptoms of cryoglobulinemia?

-Raynaud's


-Cutaneous ulcerations (esp distal extremities)


-Cold-induced urticaria (rash from cold)


-Digital gangrene

What disease?


-vascular lesions


-nectrotizing inflammation of muscular arteries


-involved artery bifurcations & branching


-Hep B antigenemia


-small & medium vessel vasculitis


Polyarteritis Nodosa (PAN)

Polyarteritis Nodosa (PAN)

ACR classification criteria for polyarteritis nodosa

-testicular pain (middle age man)*


-myalgias, weakness, or leg tenderness


-weight loss > 4 kg


-livedo reticularis


-mono/poly-neuropathy


-diastolic BP > 90


-azotemia


-Hep B


-arteriographic abnormality (aneurysm)


-biopsy containing PAN


What is wrong w this image?


What pathology?

What is wrong w this image?


What pathology?

-giant aneurysm


-arteries look like stringed sausage (not straight)



Polyarteritis Nodosa (PAN)

What disease?


(ACR Diagnosis MUST include 4/6 below)


1. Mono/poly-neuropathy


2. Eosinophils (in tissue)


3. Asthma


4. Tranistory pulmonary infiltrates


5. paranasal sinus abnormalitis


6. biopsy w/ extravascular eosinophils



-granulomatous rxn


-small & medium vessel vasculitis

Churg-Strauss syndrome (allergic angiitis & granulomatosis)

What disease?


(ACR Dx MUST include 2/4)


1. Nasal or Oral ulcers or bloody nasal drainage


2. Abnormal chest X-ray (nodules, infiltrates, cavities)


3. Urinary sediment (>5 RBC or casts)


4. granulomatous vasculitis on biopsy



-glomerulonephritis


-ELK classification


-small & medium vessel vasculitis

Wegner's granulomatous

Wegner's granulomatous usually occurs in middle-aged (NOT black) individuals w/ cough & hypertension. What Abs are associated w/ Wegners?

C-ANCA (anti PR-3)


 


(^Ab directed against neutrophils & monocytes)


 

C-ANCA (anti PR-3)



(^Ab directed against neutrophils & monocytes)


What disease?

What disease?

Wegner's Granulomatosis



(rounded granulomas on histology & intense infiltrates & cavities on x-ray)

What disease?


(ACR Dx need 3/6)


1. Age < 40 (esp young/adolescent women)


2. claudication (cramping) of extremities


3. dec brachial artery pulse


4, BP diff > 10 btwn arms


5. Bruits over subclavian or Aorta


6. Arteriogram abnorm. (narrowing/occlusion aortic arch & branches)



-more common in Asia


-medium & large vaculitis

Takayasus Arteritis (pulseless)

What are the arrows pointing at?


What disease?

What are the arrows pointing at?


What disease?

narrowing near aortic arch



Takayasu's Arteritis (pulseless disease)

What disease?


(ACR Dx need 3/5)


1. Age > 50 (at onset) (old)


2. New Cephalgia (headache)


3. Temporal Artery abn. (tender, dec pulse, risen)


4. elevated ESR > 50mm/hr (sediment rate)


5. Abn. biopsy (mono infiltrate, granulomatous inflam, usually multinuc giant cells)



-usually involves carotid artery


-scandinavian women likely


-jaw claudication or muscle pain


-medium & large vasculitis

Giant Cell (Temporal Arteritis)

Giant Cell (Temporal Arteritis)

____________ is a vasculitis w/ subcutaneous fat inflammation



associated w;


-Erythema nodosum


-Weber-Christian Disease


-Pancreatis

Panniculitis

What is the pathology?


What are the white dots?

What is the pathology?


What are the white dots?

Panniculitis


 


-fatty nodules under skin (may push up on skin & cause pain)


(purple septal areas are inflammatory cells)

Panniculitis



-fatty nodules under skin (may push up on skin & cause pain)


(purple septal areas are inflammatory cells)

What does this pt have on ant lower legs?

What does this pt have on ant lower legs?

erythema nodosum


(assoc w/ panniculits, also assoc. w/ sarcoidosis, strep, Tb)

How are vaculitides treated?

-glucocotricoids & immunosuppressives


-cyclophosphamide for severe forms

Which vasculitis involve veins (as well as arteries)?

Wegner's Granulomatosis



(H-S purpura & hypersensitivities may involve venules)