Vasculitis - Exam 2 Flashcards by Johannah Crumpton

_____ is an AI disorders characterized by inflammation of blood vessels. What organ

vasculitis

can affect any organ

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What is vasculitis characterized by? How is it classified?

Size of blood vessel typically involved
Predilection for certain organ systems
Characteristic pathologic features

Large vessel  aorta and great vessels

Medium vessel  splanchnic vessels

Small vessel  capillaries, arterioles, and venules to lungs, kidneys and skin

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What 2 vasculariritic dz tend to occur together? What specific allele?

Polymyalgia Rheumatica and Temporal Arteritis

both associated with (HLA) DR4

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Pain, stiffness in neck, shoulders, lower back, hips and thighs
Few have joint swelling
Frequently have fever, malaise, weight loss

What am I?
What will the pt complain of?

Polymyalgia Rheumatica

Trouble combing hair
Trouble putting on a coat
Difficulty rising out of a chair

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What is the tx for polymalgia rheumatica? What labs should you order? When should you start to see improvement?

Prednisone 10-20mg po daily over 2-4 weeks with slow taper

CBC, ESR/CRP

should start to see improvement within 72 hours, if no improvement -> needs to be re-evaluated

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_____ are common when tapering prednisone in a pt with polymalgia rheumatica. What is the tx? How can you monitor progress?

flares

tx flares with MTX while tapering the prednisone -> should refer to rheumatology

Can monitor progress with ESR

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What is the epidemiology of temporal arteritis? What is the mean age of onset? What alelle is it associated with?

white women over the age of 50

mean age of onset is 79

Association with HLA-DR4

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What type of vessels does temporal arteritis affect? What part specifically? What is the big artery it like to affect?

Systemic panarteritis affecting medium and large-size vessels

Proliferation of intima and fragmentation of internal elastic lamina

Involvement of one or more branches of the carotid artery but can involve the aorta and its branches

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What layer of the vessel does temporal arteritis affect? What components of the immune system play a part?

Believed to be initiated in the adventitia

Antigen driven-activated T-lymphocyte macrophages and dendritic cells play critical role

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What are the 3 layers of the blood vessel?

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headache, scalp tenderness, visual symptoms (amaurosis fugax or diplopia), jaw claudication or throat pain
HA

What am I?
Why is this an emergency?

temporal arteritis

can lead to blindness because of the anterior ischemic optic neuropathy

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What are the PE findings associated with temporal arteritis? What labs should you order? What lab value will NOT be elevated?

May have tender, thickened or nodular temporal artery

scalp or jaw pain

may have decreased pulses or bruits

ESR > 50 and often over >100, and Alkaline phosphatase may be elevated

Serum creatine kinase will NOT be elevated

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_____ is the standard of dx for temporal arteritis. What imaging could you order?

temporal artery bx

Consider CTA or MRI that may show long stretches of narrowing

US of temporal artery

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What is the tx for temporal arteritis without vision loss? What is the adjunct med?

Begin HIGH dose prednisone 40-60 mg/day for at least 1 month before tapering

consider adding ASA to reduce chance for vision loss/stroke

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When dx is Tocilizumab used in? What is the prognosis?

temporal arteritis, to reduce prolonged use of prednisone

After one year 50% have corticosteroid free remission

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What is the tx for temporal arteritis WITH vision loss?

IV methylprednisolone 1 gram daily x3 days

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What are the complications of temporal arteritis?

Ischemic optic neuropathy

CVA, scalp or tongue infarction

Subclavian artery stenosis

thoracic aortic aneurysm -> so need to screen for this

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Polyarteritis Nodosa (PAN) pts approximately 10% will also have ____

hep B

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_____ is a multisystem necrotizing arteritis involving SMALL and MEDIUM sized MUSCULAR arteries in which involvement of the renal and visceral arteries is characteristic. Does it cause muscle weakness?

Polyarteritis Nodosa (PAN)

do NOT cause muscular weakness

aka the small and medium sized muscular arteries die

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Does Polyarteritis Nodosa tend to affect the lungs?

NO!! but can affect the bronchial vessels

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What is happening in the acute stages of Polyarteritis Nodosa?

Polymorphonuclear leucocytes infiltrate ALL layers of vessel wall and perivascular areas
Causes intimal proliferation and degeneration

Mononuclear cells then infiltrate area and lesions progress and lead to necrosis

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What happens in Polyarteritis Nodosa (PAN) after the part of the vessel has died? What happens as a result?

Healing of lesions by collagen deposition that leads to further occlude vessel

Aneurysmal dilation up to 1cm in involved arteries are characteristic

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weeks to months
Fever, malaise, weight loss, headache, abdominal pain, myalgias
Fever, abdominal pain extremity pain, livedo reticularis, mononeuritis multiplex
Arthralgia, myalgia (calves) or neuropathy

What am I?
What PE finding was discussed in class?
What are the earliest specific clues that lead to this dx?

Polyarteritis Nodosa (PAN)

livedo reticularis

Combination of mononeuritis multiplex + features of systemic illness

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What are the 3 skin findings associated with polyarteritis nodosa (PAN)? What other organ is typically involved?

Combination of mononeuritis multiplex + features of systemic illness

Renal:
Arteritis without glomerulonephritis
Renal insufficiency/failure, HTN, hemorrhage from microaneurysms
Involvement of renal artery  renin-mediated HTN

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commonly presents with diffuse periumbilical pain that is precipitated by eating N/V Infarction can lead to acalculous cholecystitis, appendicitis and possibly acute abdomen What am I? What labs should you order? What will they show?

Polyarteritis Nodosa (PAN) CBC, ESR, ANCA, Rh, antinuclear antibodies CBC: anemia with leukocytosis ESR: elevated ANCA: negative low titers for rheumatoid factor or antinuclear antibodies

If you suspect PAN need to test for ______ .____ or _____ are needed for dx confirmation

hep B Tissue biopsy or angiogram needed for confirmation

What body sites should you pull your tissue bx from to confirm dx of PAN? Why?

Symptomatic sites have sensitivity ~70% and provide highest diagnostic yield Nodular skin lesions, painful testes, nerve/muscle Have the highest benefit-risk ratio

When should you order an angriogram in PAN? What do you expect to see?

If suspect with mesenteric ischemia or new onset HTN in setting of systemic illness May see aneurysmal dilations in renal, mesenteric or hepatic arteries but do NOT have to be present for dx

What is the tx for PAN? What adjunct med? What if super sick?

High dose corticosteroids (up to 60mg/day) Adding cyclophosphamide lowers risk of disease-related death and morbidity with severe disease Critically ill at presentation-pulse methylprednisolone (1g IV daily x3d)

Once the pt is in remission for PAN induced by cyclophosphamide, ___ or ___ should be used for remission. What is the 5 year prognosis rate comparing untreated to treated?

MTX or Azathioprine Untreated ~10-20% Treated 60-90%

What are poor prognostic factors for PAN? What is death usually related to?

CKD, GI ischemia, CNS dz and cardiac involvement Death is generally related to GI or cardiovascular causes

What are the complications of PAN?

GI-bowel infarction, hemorrhage Cardiovascular Cyclophosphamide treatment Glucocorticoid toxicity

What is the MC pt for granulomatosis with polyangiitis? What is another name for it?

rare in general equal sex, WHITE, mean age of onset is 40 Wegener’s

What is Granulomatosis with Polyangiitis characterized by?

Characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis Variable degrees of disseminated vasculitis involving small arteries and veins may occur

_______ hallmark is the necrotizing vasculitis of small arteries and veins together with granuloma formation; intravascular or extravascular

Granulomatosis with Polyangiitis

What is the classic triad of Granulomatosis with Polyangiitis?

Upper airway lesions Lower airway (lungs) lesions Renal lesions

______ is associated with higher relapse rates in Granulomatosis with Polyangiitis. A high percentage develop _____

Chronic nasal Staph aureus High percentage develop ANCA

Often severe upper respiratory tract findings May have saddle nose deformity Serous otitis media Subglottic tracheal stenosis may have pulm, eye, cardiac, nervous system and skin involvement Renal disease purpura Nonspecific symptoms-malaise, weakness, arthralgias, anorexia and weight loss What am I? What will the renal dz present like?

Granulomatosis with Polyangiitis Mild glomerulonephritis and proteinuria, hematuria and RBC casts

How do you dx Granulomatosis with Polyangiitis? ____ have the highest diagnostic yield

Tissue biopsy-necrotizing granulomatous vasculitis Pulmonary tissue has highest diagnostic yield

What part of the body when bx may NOT show vasculitis for Granulomatosis with Polyangiitis? What will the kidneys confirm?

Upper airway may not show vasculitis Renal can confirm pauci-immune glomerulonephritis

What lab test will 90% of pts with Granulomatosis with Polyangiitis be positive? Especially with active _____

90% have positive ANCA Specificity very high especially with active glomerulonephritis

_____ should NOT be used to assess dz activity in Granulomatosis with Polyangiitis and rise alone does NOT mean relapse

ANCA

What is the tx for Granulomatosis with Polyangiitis? What is the monitoring?

Cyclophosphamide induction with glucocorticoid and follow with gradual taper over 6-9 months CBC every 1-2 weeks

How long can you safely use cyclophosphamide in GPA tx? Why?

Limit cyclophosphamide to 3-6 months Long-term use associated with toxicity

When is Rituximab used? What do you need to screen for before starting tx?

used in the tx of GPA and with relapsing disease found to be statistically superior to cyclophosphamide so use rituximab q week x4 weeks PLUS glucocorticoids Hepatitis B-> Need to screen PRIOR to initiating treatment

What should you do for the LONG term tx of GPA? What is the 3rd line option?

Change cyclophosphamide after 3-6 months to either methotrexate or azathiprine If unable to take either-> mycophenolate mofetil

When can methotrexate NOT be given?

CANNOT be given with renal insufficiency or chronic liver disease and pregnancy

What is the tx for mild GPA? It is assumed to be severe unless stated

Not immediately life threatening or cyclophosphamide toxicity MTX + glucocorticoid

What abx has show to have some benefit in GPA to help with the sinonasal tissue? What 2 complications in GPA tend to NOT respond to systemic immunosuppressive threatment?

bactrim Subglottic tracheal stenosis and endobronchial stenosis -> may need to treat these organs specifically

How is Cyclophosphamide eliminated? What 3 things do you need to monitor for?

Monitor renal function closely infertility, DVT, PE

_____ is necrotizing vasculitis of small and medium sized arteries and veins and the is MC cause of pulm-renal sydrome

microscopic polyangiitis

_____ is diffuse alveolar hemorrhaging + glomerulonephritis that often occurs simultaneously

pulmonary-renal syndrome

What is the mean age of onset for microscopic polyangiitis? What sex?

Mean age of onset is ~57 years old Males slightly more affected than females

What is Pauci-immune nongranulomatous necrotizing vasculitis? What 2 organ systems? What lab value?

Affects small blood vessels Often causing glomerulonephritis and pulmonary capillaritis Associated with ANCA

microscopic polyangiitis overlaps with ______ and _______. What 2 organ systems?

Overlaps with polyarteritis nodosa and granulomatosis with polyangiitis (WG) Tends to affect capillaries in lungs and kidneys

How is microscopic polyangiitis different from granulomatosis with polyangiitis?

microscopic polyangiitis differentiated from WG by absence of granulomatous inflammation

Palpable “raised” purpura and other signs of cutaneous vasculitis May see fever, weight loss, musculoskeletal pain Vasculitis neuropathy, mononeuritis multiplex are common In cases of pulmonary-renal syndrome interstitial lung fibrosis is presenting condition What am I? What are some common lab findings? Does it have a destructive upper respiratory component?

microscopic polyangiitis ANCA -> Usually p-ANCA pattern May have microscopic hematuria, proteinuria, RBC casts in urine Kidney lesion NO! MP does not have a destructive upper respiratory tract disease that is seen in GPA

Segmental, necrotizing glomerulonephritis Often localized intravascular coagulation and intraglomerular thrombi on renal bx These are describing the kidney lesion for ____

Microscopic polyangiitis

Is Polyarteritis nodosa (PAN) associated with ANCA? Is MPA associated with ANCA?

PAN no ANCA MPA yes ANCA

What is the tx of microscopic polyangiitis?

tx the same as GPA Urgent induction tx with corticosteroids + either cyclophosphamide or rituximab Following remission- discontinue cyclophosphamide and initiate azathioprine, rituximab or MTX

What are the 3 complications of microscopic polyangiitis?

Pulmonary-renal syndrome Vasculitis neuropathy Renal insufficiency

_____ is the MC vasculitis in children. What time of the year? What sex?

Henoch Shönlein Purpura (HSP) Peak incidence in spring males> females

What is the etiology behind Henoch Shönlein Purpura (HSP)? What are some triggering factors?

Leucocytoclastic vasculitis with IgA deposition Incited by URIs, drugs, foods, insect bites and immunizations

What 3 things characterizes HSP?

Characterized by palpable purpura, polyarthralgia, GI s/s, glomerulonephritis may also have abdominal pain

HSP and _____ are very commonly associated. What joints are the polyarthralgias most common?

Intussusception Knees and ankles

What can HSP lead to? What will the renal bx show? ____ is usually only elevated in 1/2 of patients

CKD Bx-segmental glomerulonephritis with crescents and mesangial deposition of IgA IgA elevated in about ½ of patients

What is the tx of HSP in kiddos? What do you need to monitor these kiddos for moving forward?

Prednisone 1-2 mg/kg/day orally monitor for kidney disease and screen for proteinuria

What is the tx of severe HSP? What are the complications?

Aggressive immunosuppressants Mycophenolate mofetil CKD and bowel obstruction (due to intusseception)

ANCA is the antibody directed against certain proteins in the cytoplasmic granules of ____ and _____. What is a normal test? What do most pts with WG have?

neutrophils and monocytes normal= negative Most patients with WG have circulating autoantibodies against neutrophil cytoplasm

What are the 2 types of ANCA?

C-ANCA (cytoplasmic) P-ANCA (perinuclear)

_____ Shows diffuse, granular cytoplasmic staining pattern observed in immunofluorescence microscopy when serum antibodies bind to indicator neutrophils

C-ANCA

______ produces _____ pattern of staining in the neutrophil cytoplasm

P-ANCA perinuclear

If the ANCA test is negative, what does that tell you?

Negative-symptoms probably not due to autoimmune vasculitis

Vasculitis - Exam 2 Flashcards

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