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MSK II Test 2 > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is the pathogenesis of vasculitis?

A

Deposition of circulating immune complexes.

Activation of complement.

Damage to vessel walls.

Leukocytoclastic changes.

Necrosis & thrombosis.

Distribution & clinical symptoms determined by vessel size.

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2
Q

What are typical clinical manifestations of vasculitis in large vessels?

A

limb claudication

asymmetric BP

absence of pulses

bruits

aortic dilation

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3
Q

What are the typical clinical manifestations of vasculitis in medium sized vessels?

A

cutaneous nodules

ulcers

arthritis

livedo reticularis

digital gangrene

mononeuritis multiplex

microaneurysms

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4
Q

What are the typical manifestations of vasculitis in small vessels?

A

purpura

vesiculobullous lesions

urticaria

glomerulonephritis

alveolar hemorrhage

arhtritis

cutaneous extra-vascular necrotizing granulomas

splinter hemorrhages

uveitis

episcleritis

scleritis

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5
Q

What are the constitutional symptoms of vasculitis?

A

fever, weight loss, malaise

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6
Q

What are the diseases that cause large vessel vasculitis?

A

Large Vessel Vasculitis (LVV)

Takayasu Arteritis (TAK)

Giant Cell Arteritis (GCA)

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7
Q

What are the diseases that cause medium vessel vasculitis?

A

Medium Vessel Vasculitis (MVV)

Polyarteritis Nodosa (PAN)

Kawasaki Disease (KD)

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8
Q

What are the diseases that cause small vessel vasculitis?

A

Small Vessel Vasculitis (SVV)

ANCA-Associated Vasculitis (AAV):

Microscopic Polyangiitis (MPA)

Granulomatosis with Polyangiitis (Wegener’s) (GPA)

Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA)

Immune Complex SVV:

Anti-GBM Disease

Cryoglobulinemic Vasculitis (CV)

IgA Vasculitis (Henoch-Schönlein)(IgAV)

Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)

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9
Q

What are the diseases that cause variable vessel vasculitis?

A

Variable Vessel Vasculitis (VVV)

Behçet’s Disease (BD)

Cogan’s Syndrome (CS)- (IgG4-RD?)

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10
Q

What are the diseases that cause single organ vasculitis?

A

Single Organ Vasculitis (SOV)

Cutaneous Leukocytoclastic Angiitis

Primary CNS Vasculitis (“string of beads”)

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11
Q

What are the vasculidities that are associated with systemic disease?

A

Vasculitis Associated with Systemic Disease:

Lupus Vasculitis

Rheumatoid Vasculitis

Sarcoid Vasculitis

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12
Q

What are the vasculidities that are associated with probable etiology?

A

Hepatitis C Virus-Associated Cryoglobulinemic Vasculitis

Hepatitis B Virus-Associated Vasculitis

Syphilis-Associated Aortitis

Drug-Associated Immune Complex Vasculitis

Drug-Associated ANCA-Associated Vasculitis

Cancer-Associated Vasculitis

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13
Q

What happens to blood flow when vasculitis happens?

A

reduced blood flow d/t:

inflamed and thickened artery wall and narrowing of the lumen

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14
Q

What are the common constitutional symptoms of vasculitis?

A

fever, wt loss, fatigue, weak

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15
Q

What are the common musculoskeletal symptoms of vasculitis?

A

arthralgia/itis, myalgia.

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16
Q

What are the common cutaneous symptoms of vasculitis?

A

palpable purpura, nodules, urticaria, livedo reticularis, phlebitis, ischemia

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17
Q

What are the common pulmonary symptoms of vasculitis?

A

hemorrhage, nodules, infiltration, cavities

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18
Q

What are the common renal symptoms of vasculitis?

A

nephritis, infarction, hypertension

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19
Q

What are the common neurological symptoms of vasculitis?

A

cephalgia, mononeuritis multiplex, stroke

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20
Q

What are some common lab findings in vasculitis?

A

anemia, increased ESR, abnormal LFT, hematuria.

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21
Q

What are the causes of palpable purpura?

A

Idiopathic 50%

Infection 20% (necrotizing vasculitis)

Inflammatory 20% (leukocytoclastic vasculitis)

Medications 5%

Malignancy 5%

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22
Q

How is vasculitis treated? What is the strategy behind each drug?

A

Induce remission: Glucocorticoids – use prophylaxis for osteoporosis

Cyclophosphamide – whenever used should also use prophylaxis for Pneumocystis jiroveci.

Maintenance: AZA or MTX or mycophenolate mofetil.

Rituximab seems to be as effective as cyclophosphamide and superior to azathioprine in maintaining remission.

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23
Q

What is another name for large vessel vasculitis?

A

Large Vessel Vasculitis (Granulomatous or Giant Cell Arteritis)

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24
Q

Case 1. A 20 y/o female presents with fever, malaise, weight loss and easy fatigability of the left upper arm. She had had a left sided attack of amaurosis 2 months ago, and an exertional right calf cramping one month ago, that had been relieved by resting. On PE, vitals were normal except for BP of 160/90 in the right arm and 90/50 in the left. There was pallor of the left hand. Left carotid pulse seemed decreased with an audible bruit. There was a right femoral bruit. A 3/6 aortic diastolic murmur was present*. Sed rate was 25 mm/hr with C-RP of 30 mg/L. Right brachial index was 0.4. A carotid doppler showed extensive wall edema with marked distal stenosis in the left carotid. MRA showed carotid and axillary occlusion. Hb was 10.3 gm with SR of 20 mm/hr. This woman is most likely from:

A.Russia

B.Korea

C.Armenia

D. Israel

E.Bangladesh

A

*AR from aortic dilation since this hits the elastic arteries. Have increased BP from RAS or coarctation.

Disease is Takayasu’s arteritis

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25
Q

What is the classification criteria for Takayasu’s arteritis?

A

Age < 40 at disease onset.

Two phases – early inflammatory – late occlusive.

Decreased brachial artery pulse.

BP difference >10 between arms.

Bruits over subclavian or aorta.

Arteriogram- dilation, aneurysma or narrowing in aorta or main branches, including celiac and mesentaries.

Claudication of extremities.

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26
Q

What is another name for Takayasu’s arteritis? What is it treated with?

A

“Pulseless Disease”

Treatment: steroids

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27
Q

A 79 y/o male presents with an FUO. He has noticed pain and stiffness in the shoulders and hips for the past 12 weeks. He recalls episodes of left throat pain with chewing in the recent past. SR is 80 and WBC is normal. If the diagnosis is confirmed in this patient, it would not be surprising to find the presence of (a), (an):

A.tonsilar abscess

B.subclavian stenosis

C.aortic stenosis

D.polycythemia

E.multiple myeloma

A

subclavian stenosis

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28
Q

Which arteries are commonly affected by temporal arteritis?

A

Temporal artery

Ophthalmic artery

Facial artery

Carotid artery

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29
Q

What aortic problems are associated with temporal arteritis?

A

May have aortic aneurysms or stenosis of arch vessels,

ie aortic insufficiency murmur or bruits

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30
Q

What other condition needs to be considered with temporal arteritis? What are the SSXs?

A

Polymyalgia Rheumatica (PMR)

  • aches or pain in your shoulders
  • aches or pain in your neck, lower back, butt, hips or thighs
  • stiffness, esp in the morning
  • limited ROM in affected joints
  • less commonly, pain or stiffness in wrists or knees
  • possible low-grade fever early on, as well as faitgue, appetite loss, weight loss, depression
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31
Q

What are the SSXs of Giant Cell/Temporal arteritis? What will it show in labs and imaging?

A
  • Age > 50 at disease onset, FUO, paraneoplastic.
  • New cephalgia, jaw claudication, arm claudication, visual abnormality, neck pain, abdominal pain.
  • PMR – proximal pain and stiffness, no weakness!
  • Temporal artery abnormality (tender or decreased pulse).
  • Elevated ESR (>50mm/hr), anemia, low albumin, increased alkaline phosphatase*, thrombocytosis.
  • Abnormal artery biopsy (multinucleated giant cells).
  • CT, MRI, PET scan look for vascular edema and intimal thickening.
  • GCA related stroke usually affects the vertebral basilar territory in the extracranial location**.

*hepatic artery vasculitis or granulomatous hepatitis. The intracranial vessels have almost

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32
Q

How do you Dx suspected temporal arteritis if biopsy is normal?

A

If temporal biopsy is normal – must do MRI, 18F-FDG PET/CT, or CT angiogram for subclavian or axillary vessel narrowing.

Some have varicella-zoster vasculopathy!

Treatment - Prednisone

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33
Q

What are some essentials of Dx for temporal arteritis?

A

Granulomatous thickening of the inner portions of the branches of the external carotids

Tender temporal arteries

Jaw gets tired chewing

PE and labs are unrevealing, often have high sed rates

Easily mistaken for tension HA, patients then go blind suddenly

Most patients also have polymyalgia rheumatica, mm aches easily mistaken for rheumatism

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34
Q

What are the medium vessel vasculitidies?

A

Polyarteritis Nodosa (PAN)

Kawasaki Disease

Buergers Disease

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35
Q

This patient presents with fever, arthralgias, abdominal pain, melena, and findings as shown. He is hypertensive and has a left wrist drop and right foot drop. There is hematuria, but no hemoptysis. He has testicular tenderness. Hb is 9 gm and C-RP is moderately elevated. BUN and creatinine are mildly elevated. He is hepatitis B positive. He is ANCA negative. Angiography is positive for medium sized vessel aneurysms. He has:

A.leukocytoclastic vasculitis.

B.amyloidosis.

C.SLE.

D.WG.

E.PAN.

A

PAN

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36
Q

What are the SSXs of polyarteritis nodosa?

A

Polyarteritis Nodosa

Fever and wt. loss

Hypertension (diastolic BP > 90) – renal (renin) – unusual with small vessel vasculitis.

Livedo reticularis, skin ulcers, nodules, digital gangrene.

Can involve heart (MI or myocarditis) and brain.

Lungs spared.

Abdominal pain - periumbilical postprandial - mesenteric vessels occlusion, appendicitis, acalulous cholecystitis, etc.

Testicular pain or tenderness.

Myalgias (esp. calves), weakness or leg tenderness.

Mono (ie. foot drop) or polyneuropathy

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37
Q

What labs should be ordered for polyarteritis nodosa?

A

Azotemia, Anemia.

Hepatitis B virus.

Biopsy of skin, nerve, muscle, or testicles.

Angiogram of renal, mesenteric or hepatic artery. (microaneurysms and/or panarteritis/vasculitis)

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38
Q

What is the Tx for polyarteritis nodosa?

A

Treatment:

Induce remission:

pulse cyclophosphamide and steroids (The combination is not to be used in Hepatitis B type.)

Maintenance: azathioprine, methotrexate, mycophenolate mofetil

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39
Q

This patient has had a fever for 5 days with erythema and edema of the palms and soles, cervical adenopathy, and findings as shown. This patient has:

A. Herpes.

B. Kawasaki’s syndrome.

C. syphilis.

D. lichen planus.

E. mononucleosis.

A

Kawasaki’s Syndrome

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40
Q

What is the physician concerned about with Kawasaki’s Syndrome?

A

Development of coronary artery aneurysms

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41
Q

What is the Dx criteria for Kawasaki’s?

A

Needs: Warm (fever >5 days)

Plus, 4 of 5:

  1. Conjunctivitis - b/l and non-purulent
  2. Rash - erythematous, maculopapular, or morbilliform
  3. Erythema palsm and soles - with swelling
  4. Adenopathy, cervical - 1 unilateral node
  5. Mucous Membrane - dry, red, strawberry tongue
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42
Q

What is the treatment for Kawasaki’s Syndrome?

A
  1. High dose ASA
  2. IVIG
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43
Q

What are the complications for Kawasaki’s disease?

A

coronary artery aneurysm

myocarditis

So, do an echo!

44
Q

This 35 y/o from the middle east refuses to stop smoking. He most likely has:

A.Thromboangitis obliterans

B.FMF

C.Behcet’s disease

D.Raynaud’s disease

E.PAN

A

Thromboangitis obliterans

45
Q

What are the 2 clinical classifications of small vessel vasculitis?

A

immune complex associated

or

hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

46
Q

What is the pathophysiology of immune complex associated small vessel vasculitis?

A

Histopathology of skin with PMNs & mononuclear cells leading to fibrinoid necrosis & WBC fragments (leukocytoclastic angiitis)

47
Q

What diseases are considered hypocomplementemic urticarial small-vessel vasculitis?

A

Henoch-Schoenlein Purpura

Mixed Cryoglobulinemia

Anti-GBM

48
Q

This SLE patient has had wheals for the past 48 hours. The last episode was followed by ecchymosis. She has recently also had worsening arthritis in the wrist, hands and feet. Complement levels are low. From the above it is likely that this patient has:

A. cholinergic urticaria.

B. urticarial vasculitis.

C. type 1 IgE reaction.

D. Cullen’s sign.

E. Erythema multiforme.

A

urticarial vasculitis

49
Q

What causes urticarial vasculitis?

A

Urticaria > 24 hours = chronic.

C3a and C5a produce urticaria and neutrophilia which leads to tissue damage with release of elastase and collagenases (COPD).

These perivascular WBC infiltrates produce leukocytoclastic nuclear debris.

50
Q

What are the manifestations of urticarial vasculitis?

A

Manifestations may also consist of , fever, angioedema, Raynaud’s phenomenon, arthalgrias, uveitis, chest or abdominal pain (pleuritis), COPD, glomerulonephritis, etc.

51
Q

What are some causes of urticarial vasculitis?

A

Causes include viruses (hepatitis B or C, EB virus), malignancies, (monoclonal gammopathies, lymphomas), CTDs, and drugs (ACE inhibitors, penicillin, sulfonamides, fluoxetine, cimetidine, diltiazem, thiazides, potassium iodide, non-steroid inflammatory drugs, and glatiramer acetate).

52
Q

Which forms of urticarial vasculitis are more common?

A

Hypocomplementemic form more common with CTDs (mostly SLE, or Sjogrens), where antibodies to C1q and vascular endothelial cells are more common.

53
Q

What is the Tx for urticarial vasculitis?

A

Steroids and azathioprine. Chronic: Mycophenolate mofetil and rituximab

54
Q

This 18 year old patient on no medications and with a preceding viral prodrome presents with fever, palpable skin lesions on his legs, arthralgias, hematuria, abdominal pain and GI bleeding. What is the immunoglobulin involved?

A.IgG

B.IgA

C.IGE

D.IgM

E.IgD

A

IgA - activates alternative complement pathway

55
Q

What are the SSXs of Henoch-Schonlein purpura? Tx?

A

Purpura (100%)

Arthritis (60-85%)

Nephropathy (10-50%)

GI (85%) abdominal pain w bleeding

IgA immune complexes

Typically Children

Tx: Prednisone?

56
Q

This patient presents with hematuria, arthralgias, Raynauds phenomenon, hepatosplenomegaly and shows necrotizing vasculitis. He had sat on a cold bleacher during a football game. This is:

A.cold agglutinin disease.

B.mixed cryoglobulinemia.

C.atheroembolic disease.

D.polycythemia.

E.Buergers disease.

A

mixed cryoglobinemia - should be evaluated for Hepatitis C

57
Q

What are the clinical symptoms of cryoglobinemia?

A

Raynaud’s

Cutaneous ulceration

Cold-induced urticaria

Digital gangrene

Polyarthralgias

Purpura

Nephritis

Peripheral neuropathy - foot drop

58
Q

What is mixed cryoglobinemia associated with?

A

Associated with Hepatitis C, Sjögren’s, SLE, SBE, and osteomyelitis.

59
Q

What are some of the immune changes that lead to mixed cryoglobinemia?

A

Polyclonal mixed IgG-IgM complexes with RF activity, ie types II and III.

Type I (no RFs) is associated with a monoclonal immunoglobulin

(usually IgM as in Waldenstroms, MM or CLL, but may even be a light chain)

and cause hyperviscosity syndrome or rarely vasculitis.

60
Q

What are some of the findings associated with mixed cryoglobinemia?

A

cryocrit

leukocytoclastic vasculitis

low C4

61
Q

What are the ANCA-associated small vessel vasculitidies?

A

Granulomatosis with Polyangiitis - (Wegener’s Granulomatosis)

Microscopic polyangiitis

Eosinophilic Granulomatosis with Polyangiitis – (Churg-Strauss Disease) – Can be classified under the eosinophilic diseases with preceeding asthma and atopy, rather than ANCA associated.

62
Q

What are C-ANCA and P-ANCA Ab reacting to?

A

C-ANCA = anti-PR-3, whole cytoplasm stains

P-ANCA = anti-MPO, perinuclear staining

63
Q

What are ANCAs are associated with? What do they do?

A
  • ANCAs are associated with pauci-immune systemic necrotizing small vessel vasculitis.
  • ANCAs activate neutrophils to damage vessel walls with ultimate vasculitis and necrosis.
  • ANCA induced neutrophil activation releases factors stimulating the alternate complement pathway, which in turn draws in more neutrophils, creating a continuous loop.
  • A granulomatous reaction to extravascular necrosis may occur from extravascular neutrophils interacting with interstitial ANCAs.

*may have drug induced vasculitis with p-ANCA/MPO from PTU, hydralazine, and minocycline. Tainted cocaine may cause anti-MPO and anti-PR3.

64
Q

How do ANCAs cause vasculitis?

A

ANCAs attach to ANCA antigen on epithelials on vessel walls

Fc receptor on unprimed neutrophil reacts to ANCA

Neutrophil activated via alternative pathway activation

Neutrophils are primed with C5a and other cytokines

Acute inflammation occurs within vessel, neutrophils extravasate

Neutrophils secrete fibrin

Macrophages, monocytes, T-cells, fibroblasts and collagen are recruited and laid down

65
Q

What happens after initial vessel inflammation with neutrophils?

A

Neutrophils are primed and activated, then form a microabscess

Granulomatous reaction forms, with multinucleate giant cells and macrophages walling off abscess

66
Q

This patient whose skin lesions are palpable is positive for P-ANCA. Skin biopsy shows pauci immune nongranulomatous necrotizing vasculitis of the arterioles capillaries and venules. He has hematuria and red cell cast with some hemoptysis. Chest xray shows pneumonitis without nodules. He has a left foot drop. He has:

A. Granulomatous polyangiitis.

B. microscopic polyangitis.

C. eosinophilic granulomatous polyangiitis.

D. Henoch Schönlein purpura.

E. polyarteritis nodosa.

A

Microscopic Polyangitis

67
Q

What does microscopic polyangitis look like?

A

Looks like Wegeners (pulmonary-renal syndrome, mononeuritis, palpable purpura, etc), except no upper respiratory involvement and no granulomas!

68
Q

What Ab are involved with microscopic polyangitis?

A

P-ANCA – may be simulated by PTU, hydralazine, allopurinol, minocycline, etc.

69
Q

What is the Tx for microscopic polyangitis?

A

Treatment: Cyclophosphamide , prednisone, AZA, Rituximab

70
Q

What are 2 signs of microscopic polyangitis?

A

diffuse patchy infiltrates on CXR

circular purpura

71
Q

This patient presented with a UA showing greater than 5 RBCs per high power field and hemoptysis with nodules on chest Xray. He had a migratory oligoarthropathy in the past and has stridor upon examination. He has had a prior DVT. He was found to have a conductive hearing loss in the left ear, and the following and, thus has a, (an):

A.granulomatous polyangiitis.

B.SLE.

C.microscopic polyangiitis

D.Churg-Strauss syndrome

E.PAN

A

granulomatous polyangitis

72
Q

What are the SSXs of granulomatosis with polyangitis?

A

Nasal, Otic, Ocular, or Oral Inflammation.

Abnormal chest X-ray (nodules, fixed infiltrates, cavities –cough,

dyspnea, stridor, and or hemoptysis.

Urinary sediment (> 5rbc or casts) – RPGN*. Pulmonary-Renal Syndrome as does SLE, Goodpastures and MPA

Oligoarthritis, neuropathy, uveitis, DVTs.

73
Q

What causes Wegener’s/Granulomatosis with polyangitis?

A

Subtle presentation, lethal if missed

Caused by auto-Ab against proteinase 3

positive anti-neutrophil cytoplasm test (c-ANCA)

74
Q

What additional SSXs characterize the presentation of granulomatosis with polyangitis?

A

sore eyes

sore ears

stuffy nose

chest x-ray blip

sore joint

trace of blood in urine

destruction of the face

lung cavities and bleeds

permanent kidney failure

gangrene

death from renal failure

75
Q

What are the SSXs of Churg-Strauss Syndrome?

A

Eosinophilic Granulomatosis with polyangitis

Asthma

Eosinophilia (> 10% of WBC).

Mono or polyneuropathy.

Transitory pulmonary infiltrates.

Paranasal sinus abnormalities.

Biopsy with extravascular eosinophils.

76
Q

This Iranian patient is complaining of blurred vision as well as oral and penile pain. He reports a history of “pathergy”. This is:

A. Kawasaki disease

B. rosacea

C. SLE

D. Pemphigus

E. Behcet’s disease

A

Behcet’s Disease

apthous ulcers

retinal venulitis

77
Q

What is another name for Behcet’s?

A

Variable Vessel Vasculitis

oral-genital ulcers, pathergy

78
Q

What is Cogan’s Syndrome?

A

similar to Behcet’s, but has Chlamydia antibodies against cornea and the inner ear,

resulting in interstitial keratitis and vestibuloauditory dysfunction.

Part of IgG4 –RD.

79
Q

What causes Behcet’s?

A

Autoimmunity against heat shock proteins

HSP may result in CD4 activation without MHC I or II, and lead to TH1 activation from IL12.

The resulting T cell activation leads to increased IL8, IL17, IFN-gamma, and TNF-alpha with

resultant neutrophilia and vasculitis.

80
Q

What are some more SSXs of Behcet’s?

A

mouth ulcers (always)

eye lesions

genital ulcers

skin lesions

neurologic syndromes

infarcts of anything

thrombosis of anything

phlebitis

amyloidosis

Affects arteries and veins of all sizes = variable vessel vasculitis

81
Q

What is the epidemiology and cause of Behcet’s?

A

Epidemiology: Turkish or middle Eastern – HLA-B51.

Causes: strep sanguis (like HSP), herpes, bowel flora.

82
Q

What is the immunology behind Behcet’s?

A

Immunology: Variable vessel (arteries & veins) vasculitis involves CD4 helper T cell response through the Th1 path with production of CD8 cells, NK cells and macrophages(an IL8 disease). Thus T cells and neutrophils invade the tissues.

No autoantibodies.

83
Q

What is the classic triad of Behcet Syndrome?

A

Classic triad:

Painful apthous ulcers – mouth & genitals– 3 x in 1 year.

Pan uveitis (blindness).

84
Q

What other systems are involved in Behcet’s? What happens with them?

A

CNS involvement: seizures, meningoencephalitis, mimics multiple sclerosis.

MSK involvement: arthritis – knees & ankles.

Vascular involvement - E nodosum, phlebitis – including Budd-Chiari and dural sinus thrombosis, pathergy, arterial or aortic rupture.

GI involvement: IBD-like disease – ulcers in cecum.

GU involvement: epididymitis.

85
Q

What is the Tx for Behcet’s?

A

Steroids, AZA, chlorambucil, colchicine, interferon, thalidomide

86
Q

A 30 y/o Armenian female presents with a painful right knee, diffuse abdominal pain, and fever. She reports prior episodes, one of which led to an appendectomy. Exam shows a hot swollen right knee with an effusion. There is a rub over the right chest with inspiration. Abdominal palpation shows slight rebound tenderness. There are red raised lesion over both feet. Joint aspiration shows a WBC of 300/hpf. RF, ANA, and ASOT were negative. CRP is elevated. Urine dipstick shows 1+ proteinuria. This patient has:

A.Behcets disease

B.SLE

C.Septic arthritis

D.Sarcoidosis

E.Periodic fever syndrome

A

Periodic Fever Syndrome - FMF

Erysipelas-like plaques

Must have no other rash, oral ulcers, exudative pharyngitis, psoriasis or panniculitis

87
Q

What are the SSXs of Familial Mediterranean Fever?

A

episodic fever

arthritis

pleuritis

peritonitis

hot ankle rash

most common symptoms = abdominal pain and arthritis

proteinuria - indicates renal amyloidosis

88
Q

What is the cause of FMF?

A

Caused by lack of pyrin - a neutrophil protein that keeps them from mobbing body spaces

Pyrin = a protease that normally inactivates IL8 andC5A. Thus neutrophil cytokines yield fever.

Pyrin probably controls the inflammatory response in myelomonocytic cells at the level of the cytoskeleton organization by inhibiting NF-kB and IL-1.

A Th1 disease

89
Q

How is FMF Dx’ed?

A

Diagnosis: biopsy proven amyloid or scintography with serum amyloid protein

Don’t miss the “great mimic”. Slowing down the neutrophils with colchicine prevents the deadly secondary amyloidosis

90
Q

Why is FMF treated with colchicine?

A

Treatment of FMF = colchicine.

It is an inflammatory condition that is driven by IL-1beta, due to lack of suppression by “pyrin”

91
Q

What is single-organ vasculitis?

A

Primary Angiitis of the CNS (May be RCVS, or “string of beads” related, or true cerebral vasculitis, or amyloid angiopathy)

Cutaneous Leukocytoclastic Vasculitis (skin)

92
Q

A 38 y/o female presents with a vertebral basilar TIA. Two weeks ago she had an episode of right hemiparesis and dysphasia. She has had constant occipital headaches and periods of altered mental status. The patient has had no constitutional symptoms. Physical exam shows ataxia and slurred speech. Spinal tap shows lymphocytosis. MRI shows focal enhancing parenchymal lesions and angiogram shows reduced caliber of the left middle cerebral artery.

What is the most likely diagnosis? (no constitutional symptoms)

A. Primary angiitis of the CNS

B. Behcet’s disease

C. Infections (HIV, Hep C, Zoster, etc)

D. CTD (SLE, Sjogrens,etc)

E. Systemis vasculitis (PAN, BD, WG,etc)

A

Primary angitis of the CNS

93
Q

What does primary angitis of the CNS need to be differentiated from?

A

Primary Angitis of the CNS* (a CD4 lymphocyte disease) needs to be differentiated from:

Secondary CNS vasculitis

ie. HIV, zoster, Hep C, Behcets, PAN, cocaine, ANCA associated, etc.

Reversible Cerebral Vasoconstrictive Syndromes (PCVS/”Thunder clap headaches),

including Posterior Reversible Encephalopathy Syndrome (PRES).

94
Q

What drugs can cause cutaneous leukocytoclastic vasculitis?

A

hypersensitivity vasculitis:

Allopurinol

Antibiotics (Penicillins)

Aspirin

Cimetidine

Furosemide

Hydralazine (HLA-DRw4)

Indomethacin

Leukotriene receptor

antagonist(Zafirlukast-

Accolate)

Minocycline

Methotrexate

NSAIDs

Penacillamine

Phenytoin

PTU

Sulfasalazine

Sulfonamides

Thiazides

95
Q

What is primary angitis of the CNS?

A

A granulomatous mass replaces all or part of vessel wall

(lymphocytes, large mononuclear cells, fibroblasts, multinucleated giant cells, and variable numbers of plasma cells)

“string of beads” appearance on angiogram

96
Q

What is cutaneous leukocytoclastic vasculitis?

A

Drug reaction.

Inflammation & fibrinoid necrosis of vessel walls & cellular debris in tissue of the skin.

Pathology indistinguishable from HSP, MPA, etc.

Clinical sign is “palpable purpura”.

97
Q

What vasculitidities are associated with systemic disease?

A

Lupus Vasculitis

Rheumatoid Vasculitis

Sarcoid Vasculitis

98
Q

What vasculidities are associated with a probably etiology?

A

Hepatitis C Virus-Associated Cryoglobulinemic Vasculitis

Hepatitis B Virus-Associated Vasculitis

Syphilis-Associated Aortitis

Drug-Associated Immune Complex Vasculitis

Drug-Associated ANCA-Associated Vasculitis

Cancer-Associated Vasculitis

99
Q

If you suspect Takayasu arteritis, what studies should you do?

A

CT/MR/conventional angiography of aorta and main branches

100
Q

If you suspect primary angitis of CNS, what studies should you do?

A

MR angiography of the head

CNS lesion biopsy

101
Q

If you suspect polyarteritis nodosa, what studies should you do?

A

conventional angiography, skin biosy

102
Q

If you suspect giant cell arteritis, what studies should you do?

A

temporal artery biopsy

103
Q

If you suspect ANCA-positive vasculitidies, what studies should you do?

A

sinus, renal, lung and/or skin biopsy

104
Q

If you suspect Henoch-Schonlein purpura, what studies should you do?

A

skin and/or renal biopsy

105
Q
A

MSK II Test 2 (7 decks)

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