Ch. 36 AAV Flashcards
AAVs
(4) GPA, MPA, EGPA, Renal-limited pauci-immune GN
AAVs and associated ANCAs
EGPA: pANCA > cANCA
cANCA (PR3-ANCA): GPA>MPA>EGPA
pANCA (MPO-ANCA): MPA>GPA
Heterogenous group of antibodies that bind to antigens in the primary granules of neutrophils and lysosomes of monocytes
ANCAs
ANCAs are present in what vascuitides
AAVs
Renal-limited vasculitis
Certain drug-induced vasculitis syndromes
GPA triad
Upper respiratory tract inflamm
Lower respiratory tract inflamm
Renal disease
Genes associated with GPA
MHC Class II HLA-DP region
Environmental factors identified as triggers for GPA
Infections (S. aureus, other respi tract infections)
Drugs (PTU, hydralazine, minocycline)
Silica dust
Key effector cells of endothelial injury in GPA
Primed neutrophils activated by ANCA
Other players in the inflammatory loop of GPA besides primed neutrophils
Alternative complement pathways, autoantibodies, cellular immunity
MC features of GPA at onset
Renal > pulmonary > ENT
Limited or localized GPA is defined by
Absence of renal disease
ACR criteria for GPA
2/4
1. Nasal/oral inflammation
2. Abnormal cxr
3. Abnormal ua
4. Granulomatous inflammation
EULAR/PRINTO/PRES criteria for GPA
3/6
1. Upper airway involvement
2. LTB stenosis
3. Pulmonary involvement
4. Renal involvement
5. Granulomatous inflamm
6. ANCA positivity
Classic presentation of GPA
Pulmo hem + GN (pulmo-renal syndrome)
Condition wherein tissue diagnosis is desirable in GPA
Single organ involvement
AAV where there is pauci-immune GN and characteristic serology (elevated ANCA)
GPA
GPA vs MPA
GPA: Upper airway disease
MC clinical manifestations in children with GPA
Constitutional > Renal > Pulmonary
T/F Upper respi tract biopsy in patients with GPA have very high yield
F, disappointingly low
T/F Standard inflamm markers are helpful in determining level of disease activity in GPA
F
Useful markers to track inflammation in GPA
S100A8/A9
S100A12
Neutrophil count
Pathologic characteristic of GPA
Granulomata
T/F Presence of eosinophils in small numbers indicate EGPA instead of GPA
F, small numbers of eosinophils may be seen in GPA and EGPA is characterized by large numbers of eos
MC reported renal lesion of GPA
Renal granulomata are RARE; Extracapillary proliferation and crescent formation in a focal and segmental pattern; IF shows pauci-immune pattern
T/F GPA is usually monophasic
F, follow a chronic relapsing course
Usual duration of treatment of GPA
3-6m induction, 2-4y maintenance
Cornerstone of therapy for remission induction and maintenance in GPA
Corticosteroids