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GBA - Y4 > Harrison's 356 - Vasculitides > Flashcards

Harrison's 356 - Vasculitides Flashcards

1
Q

Vasculitis is an Inflammatory condition of blood vessels that may cause ______ in the supplied organ.

A

Vasculitis are Inflammatory condition of blood vessels that may cause Ischemia the supplied organ.

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2
Q

The damage in Vasculitis that is initiated with-

A

IC deposition on vessel wall (Type 3 HSN)

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3
Q

Following IC deposition, what are the three major possible pathological processes that cause the inflammatory damage in the different types of vasculitis?

A

1) Complement system lysis of wall
2) ANCA mediated response
3) Granuloma and T cells response

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4
Q

What are the respiratory system findings on anamnesis that should licenses adding Vasculitis on the DD?

A 
Asthma
Sinusitis
Epistaxis
Hemoptysis
Pleuritic Chest pain
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5
Q

What are the Cardiovascular and Dermatological on anamnesis findings that should licenses adding Vasculitis on the DD?

A 
Any Palpable Purpura
Mucosal Ulceration
Raynaud's Phenomena
Multiple Spontaneous Abortions
Hematuria
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6
Q

What could be common for the clinical symptoms on anamnesis of Lymphoma and Vasculitis?

A

B -Symptoms

Night Sweats, Weight Loss and Fever

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7
Q

In order to progress towards the diagnosis of Vasculitis, after we have preformed; Chest X-ray, ECG, CBC, Renal and Liver Functions, Urinalysis, Culture and Serology for Infections and Other Immune diseases. What is the next step for the definite Diagnosis?

A

Suspected Vascular Wall Biopsy

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8
Q

What are the Sensory/Neural deficits that are the indicative of possible vasculitis?

A

Mononeuritis Multiplex
Deafness
Vertigo

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9
Q

What are the main diseases on DD in Suspected Vasculitis?

A 
Bacterial Endocarditis
Vasoconstrictive Drugs (e.g. Ergot Alkaloids)
APLA
Atrial Myxoma
Atheroembolic Disease
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10
Q

What are the Large Vessel Vasculitides?

A 
Giant Cell (aka Temporal) Arteritis
Takayasu Arteritis
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11
Q

What are the Medium Vessel Vasculitides?

A

Kawasaki Disease

Polyarteritis Nodosa

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12
Q

What are the main Small Vessel Vasculitides?

A

Microscopic Polyangiitis
Granulomatosis with Polyangiitis (Wegner’s)
Eosinophilic Granulomatosis with Polyangiitis (Chrug Strauss)

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13
Q

What are the c-ANCA (PR3) associated Small Vessel Vasculitides?

A

Granulomatosis with Polyangiitis (Wegner’s)

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14
Q

What are the p-ANCA (MPO) associated Small Vessel Vasculitides?

A

Microscopic Polyangiitis

Eosinophilic Granulomatosis with Polyangiitis (Chrug Strauss)

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15
Q

What drugs may induce vasculitis?

A 
Propylthiouracil
Hydralazine
Methotrexate
Phenytoin
Allopurinol
Thiazides
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16
Q

What kind of Vasculitis is associated with a specific Very Common Nephritic Syndrome?

A

IgA Nephropathy (Berger Disease - HSP)

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17
Q

What are the pauci-immune glomerulonephritis associated Vasculitides?

A

GPA
Microscopic Polyangiitis
Eosinophilic Granulomatosis with Polyangiitis (Chrug Strauss)

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18
Q

What kind of Vasculitis is associated with Necrotizing glomerulonephritis?

A

Granulomatosis with Polyangiitis (Wegner’s)

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19
Q

What kind of vasculitis will arise especially after an HCV infection and will cause Glomerulonephritis and Peripheral Neuropathy. Additionally on lab it will be cold agglutination positive?

A

Mixed Cryoglobulinemia

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20
Q

Granulomatosis with Polyangiitis (Wegner’s)

Where will we see symptoms? What systems?

A

Respiratory and Renal Systems

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21
Q

Granulomatosis with Polyangiitis (Wegner’s)

What are the most common symptoms?

A

Sinusitis, (and even Perforation of nasal Septum)
Serous Otitis Media
Dyspnea, Pleural Effusions
Hematuria

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22
Q

Granulomatosis with Polyangiitis (Wegner’s)

What is the specific Antibody? How does it prevalence change while the disease is active or dormant?

A

c-ANCA (PR3)
90% positivity in Active GPA
60% positivity in dormant GPA

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23
Q

What is the most effective biopsy for Diagnosis of Granulomatosis with Polyangiitis (Wegner’s)?

A

Lung Biopsy

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24
Q

Granulomatosis with Polyangiitis (Wegner’s)

DD list?

A

URT Neoplasm
Cocaine addiction
Infective Granulomatous Diseases

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25
Q

Granulomatosis with Polyangiitis (Wegner’s)

Treatment in Active disease?

A

Cyclophosphamide with Prednisone for 3-6 months
than Methotrexate with Prednisone for 1-2 years
*WBC monitoring to avoid opportunistic infections

26
Q

What is a major cause of FUO in women above 50 years old? (In the context of Vasculitides)

A

Giant Cell Arteritis (aka Temporal Arteritis)

27
Q

What is the cause of ischemia of supplied organs in Giant cell Arteritis?

A

Post-Inflammatory Intimal Growth and subsequent Stenosis of vessel

28
Q

Giant Cell Arteritis (aka Temporal Arteritis)

3 Classical Symptoms?

A

Temporal/Retro-ocular Headache
Visual Disturbances
Polymyalgia Rheumatica

29
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What are the 4 major complications?

A

Ocular Neuropathy and Blindness
Claudication of Extremities
Acute Ischemia- Brain, Heart, Mesentery
Aortic Aneurysm

30
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What is the pathophysiological meaning of Weakened Limb Pulse / Claudication?

A

Involvement of Larger arteries: Subclavian, Aorta

31
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What are the laboratory findings?

A

IgG and Complement ↑
ESR↑, Anemia
ALP & GGT↑

32
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What is the laboratory value that correlates best with the tapering of the treatment?

A

ESR

33
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What is an important instruction upon preforming a biopsy for the diagnosis? why?

A

Biopsy of Temporal Artery - 3-5 cm

Segmental Lesion

34
Q

Giant Cell Arteritis (aka Temporal Arteritis)

How can we diagnose the involvement of a large vessel (Subclavian/Aorta) ? (2 steps)

A

Weakened Limb Pulse / Claudication

Indication for MRI/Angio

35
Q

Giant Cell Arteritis (aka Temporal Arteritis)

What is the treatment?

A

Prednisone 2 years - Very Responsive!
Adding Tocilizumab (Anti-IL6) is synergistic!
Aspirin for Ocular Ischemia prevention

36
Q

What vasculitis is characterized by asthma as the main symptom, mononeuritis multiplex, purpura and cardiac involvement is the one of the major causes of death ?

A

Eosinophilic Granulomatousis with Polyangitis

37
Q

What are laboratory values relevant for Churg Straus?

A

p-ANCA positive in 48% of patients
Eosinophilia
Extravscular and Systemic Granulomas on biopsy

38
Q

___________ _______ is a middle size vasculitis that involves aneurysms of systemic arteries and viceral renal arteries are the most commonly dammaged vessels.

A

Polyarthritis Nodusa is a middle size vasculitis that involves aneurysms of systemic arteries and viceral renal arteries are the most commonly dammaged vessels.

39
Q

What are the specific symptoms of PAN?

A 
ANY SYSTEM can be potentially damaged :
Renal - HTN, CRF (no hematuria) - Most Common
NMJ - Arthralgia, Peripheral Neuropathy
GI symptoms
CNS - CVA, Seizures
Cardiac - MI, CHF
Skin - Rash, Livedo Reticularis
40
Q

Microscopic Polyangitis - Definition

A

IC deposit leading to Capillary, Venular and Arteriolar Inflammation - Lung Vessels involvement and Glomerulonephritis. No Granulomas.

41
Q

Common menifastions of Microscopic Polyangitis

A

Epistaxis
Alveolar bleeding
RPGN

42
Q

Microscopic Polyangiitis

Following suspicion what are the diagnostic workup?

A

75% p-ANCA positive

Biopsy for diagnosis

43
Q

Apart from Coarctation of Aorta, what other disease is common to cause pulse deficits unilaterally? Who are the common patient?

A

Takayasu Arteritis

Young Asian Women

44
Q

_______ _________ ________ is a vasculitis that is most common to damage the skin and GI tract along with Artherlgia and Glomerulonephritis. More common in children.

A

Henoch Schonlein Purpura is a vasculitis that is most common to damage the skin and GI tract along with Artherlgia and Glomerulonephritis. More common in children.

45
Q

What is unique to the Glomerulonephritis that is common in HSP? (In children)

A

Hematuria
Proteinuria
RBC casta
Usually resolves spontaneously

46
Q

What is the main difference between children and adult HSP?

A

Renal Involvement will be more severe

Myocarditis

47
Q

What type of vasculitis damages only the skin and menifasts as purpura, macules, papules, vesicles, bullae, urticaria or ulcers?

A

ICV - Idiopathic Cutaneous Vasculitis

48
Q

a Febrile child with Congested conjectivitis, Polymorphos rash on hands and face, strewberry tongue. Suspected to have a coronary artery aneurysm. What is the most probable diagnosis?

A

Kawasaki disease

49
Q

Kawasaki disease - symptoms

“CRASH “

A 
Conjectivitis
Rash (Polymorphus)
Adenopathy (cervical)
Strewberry tongue
Hand and Foot Erythema and edema
50
Q

Unique treatment for Kawasaki disease?

A

IVIG

51
Q

What vasculitis is common to cause peripheral neuropathy, skin rash, arthritis and GN? it arises due to Immune response for diseases such as HCV, MM, Myeloproliferative disorders and causes cold agglutination.

A

Cryoglobulinemic Vasculitis

52
Q

What type of vasculitis is only possible to diagnose by brain tissue biopsy?

A

Primary CNS vasculitis

53
Q

What kind of vasculitis is classically related to Deafness, Keratitis, Vertigo and aortic valce inflammation?

A

Cogan’s Vasculitis

54
Q

What are the diagnostic lab values for HSP?

A

IgA and C3 deposits

55
Q

What drugs cause serum sickness that may involve later vasculitis?

A

Penicillin and Sulfonamides

56
Q

Whatare the infections related ti vasculitis?

A 
Histoplasma
Rickestia
EBV
HIV
HCV/HBV
57
Q

What malignant disease is associated with a Vasculitis?

A

Hairy cell leukemia with Polyarthritis Nodosa

58
Q

What is the clinical major distinction of Microscopic Polyangiitis from the other ANCA positive small Vasculitides?

A

No Involvement of Upper respiratory tract

59
Q

What is the vasculitis that is most common to occur with Polymyalgia Rheumatica?

A

Giant Cell Arteritis

60
Q

What vasculitis cannot be identified with serology and must be directly diagnosed by biopsy upon clinical suscpition?

A

Polyarteritis Nodosa

GBA - Y4 (12 decks)

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