Vasculitidies

Question 1

What are the large vessel Vasculitidies?

Answer 1

• Takayasu
• Giant Cell Arteritis
• PMR

Question 2

What are the Medium vessel Vasculitidies?

Answer 2

PAN (ANCA negative)
IgA Nephropathy
Kawasaki (Mucocutaneous lymph node syndrome)

Question 3

What are the small vessel Vasculitidies?

Answer 3

Microscopic polyangitis
EGPA
Henoch
Wegners
Leukocytoplastic

Question 4

What organs are spared in Polyarteritis Nodosa?

Answer 4

Lungs and Kidneys are spared so No Glomerulonephritis. Creatinine may be increased inflammation of the renal artery

Note:Veins are also spared

Question 5

PAN is associated with what 3 disease?

Answer 5

Hep B
Hep C
Hairy Cell Leukemia

Question 6

What neurologial features do we see in PAN?

What genital complication may happen

Answer 6

Peripheral neuropathy
Mononeuritis multiplex (wrist drop/foot drop)
LAteral leg sensory deficit due to sural nerve involvement (can be Bx the sural nerve)

Testicular pain and infarction

Question 7

How is PAN Dx? and Treated?

Answer 7

Biopsy of involed organ if thats not possible angiogram can be performed showing aneurysms

Treatment:
Steroid+Cyclophosphamide (or Rituximab) if severe (Renal, Cardiac, GI, CNS)

Mod: Steroid+MTX, Azathioprine

Assciated with Hep B/C gie Entecavir

Question 8

How does Microscopic polyangiitis differ from PAN?

How is Microscopic polyangiitis diagnosed?

Answer 8

Involves Lungs (pulm. hemorrhage) and/or Kidneys
 P-ANCA  positive

Dx: Biopsy of either lung or kidney showing pulmonary capillaritis or pauciimmune necrotizing glomerulonephritis

Question 9

How is Microscopic polyangiitis treated?

Answer 9

Prednisone+Cyclophosphamide or Rituximab when in in remission cyclophosphamide is changed to azathioprine or MTX

Question 10

What is the treatment of EGPA?

Answer 10

steroid
cyclophosphamide when there is cardiac, renal, brain or GI involvement
Mepolizumab

Question 11

What 3 disorders can give a Saddle nose deformity?

Answer 11

Wegners
Relapsing polychondritis
Syphillis

Question 12

Patients with Giant Cell Arteritis should be screened for what disease?

Answer 12

Aortic Dilatation with PET, CTA, MRI/MRA

Question 13

How is Giant Cell Arteritis treated?

Answer 13

When it is suspected, start treatment with Prednisone 60 ,g per day and send patient for biopsy, start Aspirin unless C/I

If visual symptoms are evident start high dose IV steroids

If PMR features alone low dose steroid

Question 14

What monoclonal Ab is FDA approved for Giant Cell Arteritis treatment?

Answer 14

Tocilizumab (IL-6 Ab)

Question 15

Takayasu mainly involve which artery

Answer 15

Aortic arch and its ranches, most commonly the subclavian

Question 16

How does Takayasu present?

Dx?

Rx?

Answer 16

Women of Asian or Mexican descent

presenting with bruits over vessels, claudication, MI, Stroke, TIA
-Absent peripheral pulses, discrepancy in BP or Arterial Bruits

Dx:Arteriogram

Rx: Steroid +MTX/Azathioprine

Chronic phase require vascular surgery cosultation

Question 17

What are the 3 types cryoglobulinemia?

Answer 17

Type I=Monoclonal IgG or IgM seen in multiple myeloma and Waldenstrom

Type II (Mixed)+Monoclonal IgM with RF acitivity and Polycolonal IgG associated with small vessel vasculitis-HEPATITIS C

Type III= Polyclonal IgG and IgM

Question 18

What is the diagnostic criteria for Behcet’s disease?

Answer 18

Recurrent oral ulcerations + 2 of the following

• recurrent genital ulcers
• eye lesion
• skin lesion

AND a positive pathergy test (development of sterile pustules at the sites of needle entry)

Question 19

What does the synovial fluid show in infectious arthritis?

Answer 19

its Yellow with low viscosity

WBC>50,000 although may be less with recent Rx or Abx use

Question 20

Treatment of Infectious arthritis?

Answer 20

Gram Stain (+)=Vanc
Gram stain (-)=Rocephin

If traumatic use Vanc and Rocephin

If immunocomp use Vanc + Antipseudomonal

Question 21

What is the clinical presentation of Familial MEditerranean fever Autoimmuneinflammatory syndromes)?

Answer 21

Seen in Arabs and Turks

Fever lastin1-3 days+Abdominal pain+ pleuritic chest pain+ monoarthritis of knee or ankle+ erythematous rash on the dorsum of the foot, ankle, or lower leg

Question 22

What is the mutation seen in Familial MEditerranean fever Autoimmuneinflammatory syndromes)?

What is the most serious complication?

Answer 22

• MEFV Gene in chromosome 16 that encodes Pyrin, which regulates inflammation
• most serious complication is AA amyloidosis

Question 23

How is Familial MEditerranean fever Autoimmuneinflammatory syndromes) treated?

Answer 23

Daily oral colchicine to suppress inflammation and prevent amyloidosis

Colchicine resisntant disease is treated with Anti-IL 1

Question 24

What are the common features pf Spondyloarthopathies?

Answer 24

• Inflammatory back pain that improves with exercise
• Axial and peripheral arteritis
• Enthesitis
• Dactylitis
• HLA-B27 positivity

Question 25

What are the etra-articular features of Ankylosing spondylitis?

Answer 25

- Anterior uveitis - Cardiac: Aortic regurg with symptoms of CHF and 3rd degree block - Lungs: Pulm apical bullous disease

Question 26

What 2 test are performed to assess mobility in ankylosing spondylitis?

Answer 26

Chest Expansion -Measure chest expansion during maximal inspiration and maximal forced expiration. In inspiration the chest should expand by at least 5 cm -Schober test: measures the flexion of the lumbar spine, which normally should increase by 5 cm

Question 27

Whats a feared complication of Ankylosing spondylitis?

Answer 27

Displaced spinal fracture leading to spinal cord compression usually of the c5-C6 Lumbosacral ankylosis canlead to the cauda equina sydrome

Question 28

What 2 arthritis that involves the DIP

Answer 28

``` OA Psoriatic Arthritis (+ nail pitting) ```

Question 29

what is RElapsing polychondritis and what area does it involve?

Answer 29

Episodic disorder with inflammation and destruction of cartilaginous structures -Ear=swelling,warmth, and redness of external ear with sparing of earlobe. The ear becomes soft and floppy. Middle ear involvement can lead to hearing loss and vertigo - Collapse of asal cartilage lead to saddle nose deformity - Eye-scleritis, episleritis, conjunctivitis - asymmetri nonerosive polyarthritis

Question 30

How is RElapsing polychondritis Dx and treated?

Answer 30

Dx: At least 3 of the following criteria are needed: - Bilateral auricular chondritis - Nonerosive polyarthritis - Nasal chondritis - Ocular inflammation - Respiratory tract chondritis - Cochlear or vestibular dysfunction Biopsy for definitive diagnosis Rx: NSAID for mild diease Severe: Prednisone, If patient doesn't respond to steroid then Cyclophosphamide, MTX, cyclosporine Tracheostomy, tracheal stent, vlave replacement ad aortic root replacement may be needed.