Page 418 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

392 Rami N. Al-Rohil and J. Andew Carlson

Prognosis

There are clinical and histologic predictors of the prognosis of cutaneous vasculitis that
suggest the likelihood of concomitant or later systemic vasculitis [1, 15, 68] as shown in
Table 5.

Table 5. Clinical, Pathologic, and Laboratory findings indicating a High Probability of
Systemic Disease1

Clinical signs or symptoms Suspected Systemic Vasculitis Syndrome

High fever Infection, systemic inflammatory disorders

Paresthesia, foot drop EGPA, PAN

Abdominal pain HSP, EGPA, MPA, PAN, RV, LV, BD

Frank arthritis RV, infection, PAN, systemic inflammatory

disorder

Hypertension PAN

Purpura above waist, upper extremities HSP, MPA, GPA, EGPA

> 1 type of vasculitic lesion* HSP, MPA, GPA, EGPA, RV, LV, BD

Punctate palmar lesions LV

Laboratory evaluation Suspected Systemic Vasculitis Syndrome

ESR > 40mm/hr Infection, hematologic malignancies, systemic

inflammatory disorders

Elevated RF, cryoglobulins and low complement CV

Chest x-ray: infiltrates or cavities GPA (fixed infiltrates), EGPA (non-fixed

infiltrates), MPA, malignancy

Hematuria and/or proteinuria and/or abnormal Dermal-renal vasculitis syndrome: GPA, MPA,

creatinine HSP, SLE

Hypocomplementemia UV associated with SLE, RV, CV, infective

endocarditis

Abnormal blood count Infection, hematologic malignancy, systemic

inflammatory disorders

cANCA (PR3) GPA

pANCA (MPO) MPA, EGPA

Eosinophilia, elevated IgE, elevated RF EGPA

Histologic Examination Suspected Systemic Vasculitis Syndrome

Deep dermal and/or subcutaneous small and/or Systemic vasculitis syndrome (GPA, EGPA,

muscular vessel vasculitis MPA, CV, BD, RV, LV, septic vasculitis),

malignancy associated

Palisaded neutrophilic (extravascular) GPA, EGPA, LV, RV

granulomatous dermatitis

Tissue neutrophilia SLE, infection

Tissue eosinophilia EGPA and drug induced vasculitis

Direct immunofluorescence Suspected Systemic Vasculitis Syndrome

Isolated or predominate IgA vascular deposits HSP

Lupus band (IgG, IgM, and/or C3 at the BMZ) LV, UV associated with SLE

Abbreviations: BD, Behçet disease; BMZ, basement membrane zone; c-ANCA, cytoplasmic anti-

neutrophil cytoplasmic antibody; CV, cryoglobulinemic vasculitis; EGPA, Eosinophilic

granulomatosis with polyangiitis; ESR, erythrocyte sedimentation rate; GPA, Granulomatosis with

polyangiitis; HSP, Henoch-Schonlein purpura; LV, lupus vasculitis; MPA, microscopic

polyangiitis; MPO, myeloperoxidase; PAN, polyarteritis nodosa; p-ANCA, perinuclear pattern of

anti-neutrophil cytoplasmic antibody; PR-3, antiproteinase-3; RF, rheumatoid factor; RV,

rheumatoid vasculitis; SLE, systemic lupus erythematosus; UV, urticarial vasculitis. Adapted

from [4].

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