Vasculitis Vessel size Histopatholgic Hypersensitivity type Immunoglobulin Complex formation Affected organs Diagnosis by HLA

features (mediated by)

Granulomatosis with Small/medium Neutrophils, Pathogenic T Anti-Neutrophil Paucity/lack of Vasculitis in lungs and kidneys (+ GI, skin, MSK in some). Indirect IF studies HLA-DPB1*0401.
polyangiitis (Wegener's) vessels. Monocytes, APCs, Th1 lymphocyte responses Cytoplasmic Antibodies immune complex Necrotizing and cresentic glomerulonephritis is a common (ANCA patterns) and HLA- DR4 and
(CD4+CD28-) and (ANCA)- PR3 deposits feature ELISA to identify HLA-DR13
memory T cells and specific antigens
some times plasma
cells. Also Histiocytes
and giant cells
Churg-Strauss syndrome Small/medium Esinophilia. Pathogenic T Anti-Neutrophil Paucity/lack of Lung and heart are infiltrated by eosinophils producing Indirect IF studies HLA-DRB4
vessels. Characterized by lymphocyte responses Cytoplasmic Antibodies immune complex eosinophilic pneumonia and heart failure. (ANCA patterns) and
asthma (ANCA)- MPO. deposits ELISA to identify
 IgE. Renal involvement in 50%, focal segmental specific antigens
glomerulonephritis, sometimes with crescents
Polyarteritis nodosa Small/medium Immune Present Kidney (>70%), heart, liver, gastrointestinal tract, muscle,
vessels. complex/complement peripheral nerves, rarely the lung.
mediated.
Kawasaki disease Large/medium ANCA, anti-endothelial Present Rash, erythema, cervical lymphadenopathy
vessels Abs, ICs, T cells.
Microscopic polyangiitis Small/medium No granulomatous Anti-Neutrophil Paucity/lack of Affects many organs, primarily kidneys, skin and nerves. Indirect IF studies HLA-DRB1*0901
vessels. inflammation Cytoplasmic Antibodies immune complex Crescentic Glomerulonephritis is the most serious, seen in (ANCA patterns) and t
(ANCA) -MPO deposits >90% of the cases. ELISA to identify
Lung hemorrhage is the 2nd. specific antigens.
GI Red cell casts in urine
Giant cell arteritis Large Th1 CD4+ T cell Pathogenic T
(Temporal arteritis) (+macrophage) lymphocyte responses
mediated. Adventitial
mononuclear
infiltrates. Giant cells
& patchy medial
necrosis
Takayasu's arteritis Large CD+, CD8+, HLA Pathogenic T
(pulseless disease) antigen expression, lymphocyte responses
ICAM-1, RF
Cryoglobulinemic Medium Type I, II and III. Type I is mAb, no RF Present Weakness; numbness, tingling & limb pain; kidney
vasculitis Activity. inflammation; or palpable purpura (raised, bumpy,
Types II and III are mixed reddish-purple skin rash).
IgG and IgM; IgM has RF
activity
Goodpasture’s syndrome Small Immune Complex- Between Type II and III Abs to collagen type IV Triad of pulmonary hemorrhage, glomerulonephritis Immunofluoresent ab
Mediated and anti-glomerular test (IFA)
basement membrane
Abs (anti-GBM)
Henoch-Schönlein Small Immune Complex- IgG, IgA and ICs deposits Joint pain, gastrointestinal problems and
purpura (HSP) Mediated glomerulonephritis