ANCA-Associated VasculitisVASCULITIS: 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 February 27, 2017
IgA Vasculitis (HSP) & IgA Nephropathy (Berger) February 26, 2017
Gut Vasculitis & Behcet Disease February 25, 2017
Thromboangiitis Obliterans (Buerger Disease) February 24, 2017
ANCA-Associated Vasculitis February 23, 2017
Large-Vessel Vasculitis February 22, 2017
CNS Vasculitis February 21, 2017
Marijuana Vasculitis February 20, 2017