Vasculitis syndromes

Question 1

Polymyalgia Rheumatica (PMR) general info

Answer 1

Most benign
Common age >50
Avg age 75
Females>>>males
Unknown cause

Question 2

PMR clinical presentation

Answer 2

Abrupt onset
INTENSE MORNING STIFFNESS** that can last all day
Esp seen in shoulders and hip girdle area
NO SMALL JOINT INVOLVEMENT
Normal muscle strength
Fatigue and anorexia
(15% get GCA)

Question 3

Polymyalgia Rheumatica blood work

Answer 3

Elevated CRP and ESR
Anemia in chronic dz
Elevated platelets

Question 4

PMR treatment

Answer 4

Low dose steroids (10-20 mg/day)
prednisone is drug of choice

This should bring CRP and ESR back to normal

Question 5

Long term tx of Polymalgia Rheumatica

Answer 5

Usually self limiting
65% able to taper off by 1 year
>85% in 2 years

Question 6

Giant cell arteritis General info

Answer 6

Can occur by itself or along with PMR
Rare
Age >50
involves medium/large blood vessels of the head and neck including the blood vessels that supply the optic nerve

Question 7

GCA pathophysiology

Answer 7

Unknown trigger causes inflam response with the release of IL1 and IL6
Infiltration of inflam cells into adventitia of temporal arteries
Giant cell histologic pattern

Question 8

GCA clinical presentation

Answer 8

Scalp tenderness
Temporal headaches 
Jaw claudication
Sudden loss of vision 
Bounding or absent temporal artery pulses

Question 9

GCA diagnostic studies

Answer 9

temporal arty biopsy=gold standard*
Elevated ESR and CRP (usually higher than PMR levels)
Anemia

Question 10

GCA treatment

Answer 10

High dose prednisone*** 60 mg/day
Slow taper over 1-2years
Some pts require chronic low dose (10mg/day)

Question 11

Possible giant cell arteritis complications

Answer 11

Blindness
Scalp necrosis
Lingual infarction
Aortic dissection/aneurysm

Question 12

Complications from high dose steroid use

Answer 12

Osteoporosis
Cataracts
Elevated blood sugars
Weight gain

Question 13

Granulomatosis with polyangiitis (GWP) general info

Answer 13

Potentially fatal vasculitis involving SMALL VESSELS
Pathology shoe necrotizing granulomas usually in upper airway, lungs and kidneys

Rare

Question 14

Vasculitis

Answer 14

Inflammation of blood vessel walls leading to altered blood flow thru obstructed walls
*ischemia and tissue damage
*intense inflammatory rxn
CAN BE FATAL

Question 15

GWP pathophysiology

Answer 15

C-ANCAs play a central role
These auto antibodies interact with primed neutrophils to cause vascular injury and necrosis

Histologic lesions show granulomas

Question 16

GWP clinical presentation in eyes

Answer 16

Epsicleritis/scleritis

Proptosis due to retro orbital mass

Question 17

GWP presentation in CNS

Answer 17

Rare mass lesion

Question 18

GWP presentation in upper airway

Answer 18

Otitis media 
Nasal chondritis
Sinusitis w purulent discharge and epistaxis 
Ulcerations 
Subglottic stenosis

Question 19

GWP kidney presentation

Answer 19

Neprotic syndrome
Proteinuria
Renal failure

Question 20

GWP skin presentation

Answer 20

Palpable purpura due to leukocytoclastic vasculitis
Pyoderma gangrenosum
Panniculitis

Question 21

GWP lung presentation

Answer 21

Cough
Hemoptysis
Hemorrhage
Resp failure

Question 22

GWP cardiac presentation

Answer 22

Pericarditis

Conduction abnormalities

Question 23

GWP systemic presentation

Answer 23

Fever
Night sweats
Wt loss
Fatigue

Question 24

GWP and cANCA

Answer 24

Presence of c-ANCA
(Positive 80-90% of time in generalized GWP)

*c-ANCA often negative when GWP limited to airways

Question 25

GWP lab results

Answer 25

``` Elevated ESR and CRP Anemia Leukocytosis Thrombocytosis Elevated Cr Hematuria Proteinuria ```

Question 26

GWP treatment

Answer 26

High dose steroids and Cyclophosphamide**** | Methotrexate or Azathioprine sometimes used as steroid sparing agents

Question 27

Polyarteritis nodosa (PAN) general info

Answer 27

Medium vessel vasculitis Can be caused by Hep B Peak incidence 50s and 60s Slightly more common in males

Question 28

Polysrteritis nodosa (PAN) pathophysiology

Answer 28

In Hep B assoc cases...immune complexes play role | Not fully understood in non Hep B cases

Question 29

PAN clinical presentation

Answer 29

Fever, fatigue, wt loss Abdominal pain due to mesenteric angina/ischemia Mononeuritis multiplex Myalgias/arthalgias/mild arthritis Skin-livedo reticularis, palpable purpura, fingertip ulcerations subcutaneous nodules Testicular tenderness

Question 30

Complications of PAN

Answer 30

Chronic renal failure Bowel perforation Stroke/cerebral hemorrhage due to HTN Foot/wrist drop

Question 31

Labs of PAN

Answer 31

``` Elevation of ESR and CRP Absence of ANCA Elevated transaminases Decreased albumin Proteinuria Hematuria (no casts) ```

Question 32

Imaging studies of PAN

Answer 32

Mesenteric and or renal angiography***** | Biopsies seldom done

Question 33

PAN treatment

Answer 33

High dose steroids cyclophosphamide Methotrexate or Azathoprine used as steroid sparing agents once dz is under control (Tx hep B w anti virals)