Vasculitis Syndromes Polymyalgia - PowerPoint Presentation

Slide1

Vasculitis Syndromes

Polymyalgia

Rheumatica,Giant

Cell

Arteritis

, Wegener’s

Granulomatosis

,

Polyarteritis

Nodosa

Slide2

What is Vasculitis?

Disease characterized by inflammation of blood vessel walls, leading to altered blood flow through obstructed walls. This causes ischemia and tissue damage.

In addition there is an intense inflammatory

rxn

causing further systemic signs and symptoms

Can be fatal

Slide3

You Should Suspect Vasculitis

Unexplained signs and

sxs

Multisystem disease

Unexplained elevated ESR/CRP

Skin lesions (palpable

purpura

)

Ischemic vascular changes (

Raynaud’s

, gangrene,

livedo

,

claudication

)

Glomerulonephritis

Mononeuritis

multiplex

Intestinal angina

Inflammatory ocular

diease

Arthalgias

/arthritis,

myalgias

Sudden visual loss/headache

Slide4

Select Vasculitides

Polymyalgia

Rheumatica

Giant Cell or Temporal

Arteritis

Wegener’s

Granulamatosis

Polyarteritis

Nodosa

Slide5

Polymyalgia Rheumatica (PMR)

Most ‘benign‘ of the group

Common: 50/100,000, age > 50, average age 75. Highest prevalence in northern European ancestry, females>>males

Cause unknown

Slide6

PMR Clinical Presentation

Usually abrupt onset

Intense morning stiffness and pain that can last all day involving the shoulders and hip girdle area

No small joint involvement

Muscle strength normal

Fatigue and anorexia common

Elevated CRP and ESR; anemia of chronic disease, elevated platelets

15% get GCA (more later)

Slide7

PMR Treatment

Low Dose Steroids (10-20 mg/day)

The only drug that works

Look to normalize the CRP and ESR; if they continue to be elevated, rethink the

dx

(?

paraneoplastic

syndrome or GCA)

Usually self-limited: 65% of patients able to taper off Prednisone by 1 year, >85% in 2 years

Disease flares not uncommon as prednisone is tapered and may require dose adjustments

Slide8

Giant Cell Arteritis

Can occur exclusively but often seen with PMR

Rare: 15/100,000

Age >50

Cause unknown

Involves the medium/large blood vessels of the head and neck including the blood vessels that supply the optic nerve

Slide9

GCA Pathophysiology

Unknown trigger causes inflammatory response with the release of IL-1 and IL-6.

This leads to systemic symptoms and the infiltration of inflammatory cells into the adventitia of the temporal and other involved arteries

Typical

histologic

pattern: Giant Cells

Slide10

Slide11

GCA Clinical Presentation

Variable

Scalp tenderness

Temporal headaches

Jaw

Claudication

Sudden loss of vision

+/- PMR

sxs

Rare- upper extremity

claudication

due to

subclavian

involvement

Constitutional

sxs

: FUO, wt loss, fatigue

Bounding OR absent temporal artery pulses

Rarely

subclavian

bruits

Slide12

Slide13

GCA Diagnostic Studies

Temporal Artery Biopsy is the gold standard

Elevated ESR and CRP, usually levels higher than in PMR

Anemia

Elevated LFTs not uncommon

Slide14

Treatment of GCA

High dose Steroids (60 mg/day) is the only drug that works

Slow taper over time usually 1-2 years. Some patients require low dose (<10 mg/day) chronically

Slide15

GCA Complications

Blindness

Scalp Necrosis

Lingual Infarction

Aortic Dissection/Aneurysm

Complications from high dose steroids: osteoporosis, cataracts, elevated blood sugars, wt. gain etc.

Slide16

Wegener’s Granulomatosis (WG)

Potentially fatal

vasculitis

involving small vessels

Rare: 3-14/million, more common in whites, any age but rare in children

Pathology shows necrotizing

granulomas

usually in upper airways, lungs and kidneys

Slide17

WG Pathophysiology

Complex

immunopathogenic

events in which the production and activity of ANCAs (usually c-ANCA) play a central role. These

autoantibodies

interact with primed

neutrophils

to cause vascular injury and necrosis.

Histologic

lesions show

granulomas

Slide18

WG Clinical Presentation

Variable, multisystem involvement

Organs:

Eyes

:

episcleritis

/

scleritis

,

proptosis

due retro-orbital mass

CNS

: rare mass lesion

Upper airway

:

otitis

media, nasal

chondritis

, sinusitis with purulent drainage and

epistaxis

, ulcerations,

subglottic

stenosis

Kidney

:

neprotic

syndrome,

proteinuria

, renal failure

Skin

: palpable

purpura

due to

leukocytoclastic

vasculitis

,

pyoderma

gangrenosum

,

panniculitis

L

ung

: cough,

hemoptysis

, hemorrhage,

resp

failure

Cardiac

:

pericarditis

, conduction abnormalities

Systemic

: fever, night sweats, wt loss, fatigue

Slide19

Palpable

Purpura

Slide20

Eye Involvement

Slide21

Face Involvement

Slide22

WG Diagnostic Studies

Presence of

c-ANCA

(

cytoplasmic

staining pattern

antineutrophil

cytoplasmic

antibodies + clinical picture is often enough to make the diagnosis. It is + 80-90% of generalized WG.

If the c-ANCA is -, tissue biopsy of lung or kidney is recommended.

“Limited” refers to disease limited to the airways; c-ANCA often is -.

Slide23

Additional labs

Elevated CRP and ESR

Anemia,

leukocytosis

, &

thrombocytosis

Elevated Cr

Active urine sediment with red cell casts,

hematuria

and

proteinuria

Slide24

WG Clinical Course/Progression

Prior to

immunosuppression

therapies, WG was uniformly fatal. Now survival rates almost 90% with aggressive treatment.

High dose steroids and

Cyclophosphamide

are cornerstone of therapy.

Methotrexate

or

Azathioprine

sometimes used as steroid sparing agents.

Slide25

Polyarteritis Nodosa (PAN)

Medium vessel

vasculitis

Can be caused by

Hep

B

5/million cases

Peak incidence 50’s & 60’s, slightly more common in males

Slide26

PAN Pathophysiology

In

Hep

B

assoc cases immune complexes play significant role

In non

Hep

B cases, the

pathophysiology

is less understood

Slide27

PAN Clinical Presentation

Systemic

: fever, fatigue, wt loss

Abdominal pain

due to mesenteric angina/ischemia

Mononeuritis

multiplex

Myalgias

/

arthalgias

/mild arthritis

Hypertension

Skin

:

livedo

reticularis

, palpable

purpura

, fingertip ulceration, subcutaneous nodules

Testicular

pain or tenderness

Slide28

Livedo

reticularis

Slide29

Subcutaneous Nodules

Slide30

Complications of PAN

C

hronic renal failure

Bowel perforation

Stroke/cerebral hemorrhage due to HTN

Foot/wrist drop

Slide31

Wrist Drop

Slide32

Labs of PAN

Elevation of acute phase reactants (ESR, CRP etc)

Absence of ANCA

Elevated

transaminases

, decreased albumin

+/-

Hep

B

Urine:

proteinuria

and

hematuria

without casts

Slide33

Imaging Studies of PAN

Mesenteric and/or renal angiography is the test of choice

Biopsies seldom done

Slide34

Angiogram

Slide35

PAN Treatment

High dose steroids and

Cyclophosphamide

Methotrexate

or

Azathioprine

is used as steroid sparing agents later once the disease is controlled

Treatment for

Hep

B with

antivirals

. Sometimes plasma exchange is used to remove immune complexes