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Rheumatology > Vasculitidies > Flashcards

Vasculitidies Flashcards

1
Q

What are the large vessel Vasculitidies?

A
  • Takayasu
  • Giant Cell Arteritis
  • PMR
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2
Q

What are the Medium vessel Vasculitidies?

A

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

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3
Q

What are the small vessel Vasculitidies?

A 
Microscopic polyangitis
EGPA
Henoch
Wegners
Leukocytoplastic
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4
Q

What organs are spared in Polyarteritis Nodosa?

A

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

Note:Veins are also spared

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5
Q

PAN is associated with what 3 disease?

A

Hep B
Hep C
Hairy Cell Leukemia

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6
Q

What neurologial features do we see in PAN?

What genital complication may happen

A 
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

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7
Q

How is PAN Dx? and Treated?

A

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

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8
Q

How does Microscopic polyangiitis differ from PAN?

How is Microscopic polyangiitis diagnosed?

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

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

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9
Q

How is Microscopic polyangiitis treated?

A

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

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10
Q

What is the treatment of EGPA?

A

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

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11
Q

What 3 disorders can give a Saddle nose deformity?

A

Wegners
Relapsing polychondritis
Syphillis

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12
Q

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

A

Aortic Dilatation with PET, CTA, MRI/MRA

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13
Q

How is Giant Cell Arteritis treated?

A

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

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14
Q

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

A

Tocilizumab (IL-6 Ab)

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15
Q

Takayasu mainly involve which artery

A

Aortic arch and its ranches, most commonly the subclavian

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16
Q

How does Takayasu present?

Dx?

Rx?

A

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

17
Q

What are the 3 types cryoglobulinemia?

A

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

18
Q

What is the diagnostic criteria for Behcet’s disease?

A

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)

19
Q

What does the synovial fluid show in infectious arthritis?

A

its Yellow with low viscosity

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

20
Q

Treatment of Infectious arthritis?

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

If traumatic use Vanc and Rocephin

If immunocomp use Vanc + Antipseudomonal

21
Q

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

A

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

22
Q

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

What is the most serious complication?

A
  • MEFV Gene in chromosome 16 that encodes Pyrin, which regulates inflammation
  • most serious complication is AA amyloidosis
23
Q

How is Familial MEditerranean fever Autoimmuneinflammatory syndromes) treated?

A

Daily oral colchicine to suppress inflammation and prevent amyloidosis

Colchicine resisntant disease is treated with Anti-IL 1

24
Q

What are the common features pf Spondyloarthopathies?

A
  • Inflammatory back pain that improves with exercise
  • Axial and peripheral arteritis
  • Enthesitis
  • Dactylitis
  • HLA-B27 positivity
25
Q

What are the etra-articular features of Ankylosing spondylitis?

A
  • Anterior uveitis
  • Cardiac: Aortic regurg with symptoms of CHF and 3rd degree block
  • Lungs: Pulm apical bullous disease
26
Q

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

A

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

27
Q

Whats a feared complication of Ankylosing spondylitis?

A

Displaced spinal fracture leading to spinal cord compression usually of the c5-C6

Lumbosacral ankylosis canlead to the cauda equina sydrome

28
Q

What 2 arthritis that involves the DIP

A 
OA
Psoriatic Arthritis (+ nail pitting)
29
Q

what is RElapsing polychondritis and what area does it involve?

A

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
30
Q

How is RElapsing polychondritis Dx and treated?

A

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.

Rheumatology (4 decks)

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