Shanley's notes on Vasculitis

Question 1

What are the classic features of polymyalgia Rheumatica?

Answer 1

1. mostly in >50 y.os
2. Pain+ stiffness in shoulders and necks for >4wks
3. High EsR>40mm/hr
4. No other relevant diagnosis, except Giant Cell Arteritis
5. Rapid response to steroids

Question 2

What is Vasculitis?

Answer 2

Destructive Inflammatory process primarily affecting arteries and or veins. May be from direct vascular infection or from idiopathic causes.

Question 3

Vasculitis may arise from direct vascular infection like?

Answer 3

1. Septic emboli from infective endocarditis
2. Disseminated Gonococcal infection
3. Angioinvasion by Aspergillus or Mucor
4. Syphilitic Aortitis (thoracic aorta in tertiary Syph.)
5. Rocky Mt spotted fever
6. Varicella-Zoster virus in vessel walls (primary vasculitis of the CNS)

Question 4

What conditions commonly mimic vasculitis?

Answer 4

1. Thromboelmbolic conditions
2. Direct Vascular injury (radiation, hypertension, AMYLOIDOSIS,..)
3. Cancer associated symptoms
4. Vasoplastic drug effects (coccaine, amphetamines,..)
5. Vasoplastic drug effect
6. Vascular Malformation (coartctaion of the aorta, fibromusc dysplasia)

Question 5

POSITIVE CONGO RED!!!

Answer 5

AMYLODOSIS!

Question 6

What protein is present in ALL amyloidosis cases?

Answer 6

Serum Amyloid P component (SAP)

Question 7

What are the major types of Amyloidosis?

Answer 7

1. AL -amyloid (in plasma cell dyscrasias)
2. AA-amyloidosis (in Chronic Inflammatory conditions)
3. Familial Amyloidosis(most commonly involving transthyretin mutations)

Question 8

Large Vessel Vasculitis differential DDx includes?

Answer 8

1. Takayasu Arteritis: Granulomatous “pulseless disease” in young asians.
2. Giant Cell Arteritis

Question 9

Medium vessel arteristis differential DDX includes?

Answer 9

1. Polyarteritis Nodosa: Lung sparing necrotizing vasculitis. HTN classic coz of renal artery involvement.
2. Kawasaki disease: Kids < 4 yrs; coronary artery involvement

Question 10

ANCA- Associated small vessel Vasculitis differential DDX includes?

Answer 10

1. Microscopic Polyangitis;Necrotising Vasculitis (p-ANCA) - NO granuloma, or nasopharyngeal symptoms.
2. Wegener’s :Necrotizing granulomatous inflammation (c-ANCA).Nasopharynx(Sinuses), lungs and Kidneys.
3. Churg-Strauss:Necrotizing granulomatous inflammation– Eosinophilic Granulomatosis w/ polyangitis

Question 11

Immune Complex small vessel vasculitis differential DDx includes?

Answer 11

1. Cyroglobulinemia Vasculitis
2. IgA Vasculitis (Henoch Schonlein); esp in kids!!
3. Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)

Question 12

What are the classic features of Sjögren’s syndrome?

Answer 12

Antibodies to Ro (SS-A) or La (SS-B)
I. Symptomatic xerostomia for >3 months
II Symptomatic dry eyes for >3 months
III Positive Schirmer I test ≤5 mm/5 min or rose Bengal score
IV Abnormal labial gland biopsy
V Positive result for unstimulated whole salivary flow (≤1.5 mL in 15 min) or abnormal parotid sialography or salivary scintigraphy