Vasculitidies Flashcards
What are the large vessel Vasculitidies?
- Takayasu
- Giant Cell Arteritis
- PMR
What are the Medium vessel Vasculitidies?
PAN (ANCA negative)
IgA Nephropathy
Kawasaki (Mucocutaneous lymph node syndrome)
What are the small vessel Vasculitidies?
Microscopic polyangitis EGPA Henoch Wegners Leukocytoplastic
What organs are spared in Polyarteritis Nodosa?
Lungs and Kidneys are spared so No Glomerulonephritis. Creatinine may be increased inflammation of the renal artery
Note:Veins are also spared
PAN is associated with what 3 disease?
Hep B
Hep C
Hairy Cell Leukemia
What neurologial features do we see in PAN?
What genital complication may happen
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
How is PAN Dx? and Treated?
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
How does Microscopic polyangiitis differ from PAN?
How is Microscopic polyangiitis diagnosed?
Involves Lungs (pulm. hemorrhage) and/or Kidneys P-ANCA positive
Dx: Biopsy of either lung or kidney showing pulmonary capillaritis or pauciimmune necrotizing glomerulonephritis
How is Microscopic polyangiitis treated?
Prednisone+Cyclophosphamide or Rituximab when in in remission cyclophosphamide is changed to azathioprine or MTX
What is the treatment of EGPA?
steroid
cyclophosphamide when there is cardiac, renal, brain or GI involvement
Mepolizumab
What 3 disorders can give a Saddle nose deformity?
Wegners
Relapsing polychondritis
Syphillis
Patients with Giant Cell Arteritis should be screened for what disease?
Aortic Dilatation with PET, CTA, MRI/MRA
How is Giant Cell Arteritis treated?
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
What monoclonal Ab is FDA approved for Giant Cell Arteritis treatment?
Tocilizumab (IL-6 Ab)
Takayasu mainly involve which artery
Aortic arch and its ranches, most commonly the subclavian
How does Takayasu present?
Dx?
Rx?
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
What are the 3 types cryoglobulinemia?
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
What is the diagnostic criteria for Behcet’s disease?
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)
What does the synovial fluid show in infectious arthritis?
its Yellow with low viscosity
WBC>50,000 although may be less with recent Rx or Abx use
Treatment of Infectious arthritis?
Gram Stain (+)=Vanc Gram stain (-)=Rocephin
If traumatic use Vanc and Rocephin
If immunocomp use Vanc + Antipseudomonal
What is the clinical presentation of Familial MEditerranean fever Autoimmuneinflammatory syndromes)?
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
What is the mutation seen in Familial MEditerranean fever Autoimmuneinflammatory syndromes)?
What is the most serious complication?
- MEFV Gene in chromosome 16 that encodes Pyrin, which regulates inflammation
- most serious complication is AA amyloidosis
How is Familial MEditerranean fever Autoimmuneinflammatory syndromes) treated?
Daily oral colchicine to suppress inflammation and prevent amyloidosis
Colchicine resisntant disease is treated with Anti-IL 1
What are the common features pf Spondyloarthopathies?
- Inflammatory back pain that improves with exercise
- Axial and peripheral arteritis
- Enthesitis
- Dactylitis
- HLA-B27 positivity