Vasculitis

VASCULITIS: CLASSIFICATION AND COMPARISONS

Three tables below compare the diagnoses according to the size vessel involved (determined clinically or by histology or imaging)

Small Vessel Vasculitis

Diagnosis (by vessel size) Demographic Features  Local Findings Systemic Findings Diagnostic Test(s)a Treatment
Leukocytoclastic (hypersensitivity) vasculitis M = F; all ages can be affected Palpable purpura; less commonly – superficial erosions, bullae, urticaria Associated with HSP, cryo-globulinemia, RA, SLE, PAN, GPA, Churg-Strauss, PBC, ulcerative colitis, drugs, infection (gono-coccus, meningococcus, staphylococcus, SBE, HBV, HCV, CMV, EBV), neoplasia (Hodgkin’s, lymphoma, leukemia, myeloma) Skin Bx is diagnostic Treat underlying disorder; seldom requires the use of steroids, colchicine or dapsone
Henoch Schonlein purpura  M = F; children (2-11 yrs), adults (30-70 yrs) Palpable purpura of the lower extremities and buttocks; diarrhea, cramping, intussusception Renal failure, arthralgia,
arthritis, fever
 IgA deposition on skin,GI or renal Bx Self-limiting in most and will only require supportive care; steroids are not effective and are reserved for CNS, testicular, intestinal or joint disease
Hypocomplementemic urticarial vasculitis  F > M; usually
young adults
(range, 20-60
years)
Recurrent or chronic
urticaria, palpable
purpura
Arthralgias, arthritis, abdominal pain, N/V, fever,
lymphadenopathy
Dx by skin Bx; low C3, C4, CH50; ↑ESR, RF negative,
some have
low-titer ANA
Most are treated with NSAIDs or prednisone; hydroxychloroquine or cytotoxic therapy is seldom needed
Microscopic polyangiitis  M > F; middle-aged and elderly adults (range,
40-60 years)
 Palpable purpura,
hemoptysis,
abdominal pain,
hematochezia,
neuropathy,
crescentic GN
Arthralgia, myalgia, fever Dx by tissue Bx; 40% cANCA+; 60% pANCA+; ↑ESR  High dose prednisone and CTX

 

Small and Medium-Size Vasculitis

Diagnosis (by vessel size) Demographic Features  Local Findings Systemic Findings Diagnostic Test(s)a Treatment
Leukocytoclastic (hypersensitivity) vasculitis M = F; all ages can be affected Palpable purpura; less commonly – superficial erosions, bullae, urticaria Associated with HSP, cryo-globulinemia, RA, SLE, PAN, GPA, Churg-Strauss, PBC, ulcerative colitis, drugs, infection (gono-coccus, meningococcus, staphylococcus, SBE, HBV, HCV, CMV, EBV), neoplasia (Hodgkin’s, lymphoma, leukemia, myeloma) Skin Bx is diagnostic Treat underlying disorder; seldom requires the use of steroids, colchicine or dapsone
Henoch Schonlein purpura  M = F; children (2-11 yrs), adults (30-70 yrs) Palpable purpura of the lower extremities and buttocks; diarrhea, cramping, intussusception Renal failure, arthralgia,
arthritis, fever
 IgA deposition on skin,GI or renal Bx Self-limiting in most and will only require supportive care; steroids are not effective and are reserved for CNS, testicular, intestinal or joint disease
Hypocomplementemic urticarial vasculitis  F > M; usually
young adults
(range, 20-60
years)
Recurrent or chronic
urticaria, palpable
purpura
Arthralgias, arthritis, abdominal pain, N/V, fever,
lymphadenopathy
Dx by skin Bx; low C3, C4, CH50; ↑ESR, RF negative,
some have
low-titer ANA
Most are treated with NSAIDs or prednisone; hydroxychloroquine or cytotoxic therapy is seldom needed
Microscopic polyangiitis  M > F; middle-aged and elderly adults (range,
40-60 years)
 Palpable purpura,
hemoptysis,
abdominal pain,
hematochezia,
neuropathy,
crescentic GN
Arthralgia, myalgia, fever Dx by tissue Bx; 40% cANCA+; 60% pANCA+; ↑ESR  High dose prednisone and CTX

 

Large Vessel Vasculitis

Diagnosis (by vessel size) Demographic Features  Local Findings Systemic Findings Diagnostic Test(s)a Treatment
Leukocytoclastic (hypersensitivity) vasculitis M = F; all ages can be affected Palpable purpura; less commonly – superficial erosions, bullae, urticaria Associated with HSP, cryo-globulinemia, RA, SLE, PAN, GPA, Churg-Strauss, PBC, ulcerative colitis, drugs, infection (gono-coccus, meningococcus, staphylococcus, SBE, HBV, HCV, CMV, EBV), neoplasia (Hodgkin’s, lymphoma, leukemia, myeloma) Skin Bx is diagnostic Treat underlying disorder; seldom requires the use of steroids, colchicine or dapsone
Henoch Schonlein purpura  M = F; children (2-11 yrs), adults (30-70 yrs) Palpable purpura of the lower extremities and buttocks; diarrhea, cramping, intussusception Renal failure, arthralgia,
arthritis, fever
 IgA deposition on skin,GI or renal Bx Self-limiting in most and will only require supportive care; steroids are not effective and are reserved for CNS, testicular, intestinal or joint disease
Hypocomplementemic urticarial vasculitis  F > M; usually
young adults
(range, 20-60
years)
Recurrent or chronic
urticaria, palpable
purpura
Arthralgias, arthritis, abdominal pain, N/V, fever,
lymphadenopathy
Dx by skin Bx; low C3, C4, CH50; ↑ESR, RF negative,
some have
low-titer ANA
Most are treated with NSAIDs or prednisone; hydroxychloroquine or cytotoxic therapy is seldom needed
Microscopic polyangiitis  M > F; middle-aged and elderly adults (range,
40-60 years)
 Palpable purpura,
hemoptysis,
abdominal pain,
hematochezia,
neuropathy,
crescentic GN
Arthralgia, myalgia, fever Dx by tissue Bx; 40% cANCA+; 60% pANCA+; ↑ESR  High dose prednisone and CTX

Adapted from: RheumaKnowledgy


Musings

Miscellaneous Vasculitides

IgA Vasculitis (HSP) & IgA Nephropathy (Berger)

Gut Vasculitis & Behcet Disease

Thromboangiitis Obliterans (Buerger Disease)

ANCA-Associated Vasculitis

Large-Vessel Vasculitis

CNS Vasculitis

Marijuana Vasculitis