WORKUP FOR VASCULITIS

By Gary M. White, MD

Vasculitis causing necrotic leg ulcers Courtesy Michael O Murphy MD


Vasculitis is defined as inflammation of blood vessels. Inflammation, damage, occlusion and necrosis may all result. The vasculitides have traditionally been categorized by the size of the vessel involved: small, medium, mixed (small and medium) and large.

Clinical Presentation and Categorization of Vasculitides

Vessel Size Clinical Diseases
Small Palpable purpura, urticarial lesions, small ulcers may occur, but less often Classic (may be due to a variety of antigens including drug, infection), Henoch-Schönlein Purpura, Acute Hemorrhagic Edema of Infancy, Urticarial Vasculitis, Cryoglobulinemia, Erythema Elevatum Diutinum, Granuloma Faciale
Medium Livedo, purpura, necrosis, ulcers, mononeuritis multiplex, necrosis of major organs Polyarteritis Nodosa
Mixed (small and medium vessels) see above ANCA associated (Microscopic Polyangiitis, Granulomatosis with Polyangiitis, Churg-Strauss Syndrome), Septic vasculitis
Large Necrosis, ulcers, claudication Temporal Arteritis, Takayasu arteritis

Of note, small artery disease may give rise to ulcers and subcutaneous nodules.

A history and physical with emphasis on consideration of any new drugs, signs of infection (e.g. sore throat, fever, sinusitis, dental symptoms), OTC medications, lymph node exam, a skin biopsy (the best lesion to biopsy is well formed papule about 24 hours old. Biopsying too early will result in a non-diagnostic biopsy). One may obtain a skin biopsy for direct immunoflourescence (e.g. helpful in Henoch-schonlein purpura in adults).

See also necrosis.

Laboratory

CBC with peripheral eosinophil count (Churg-strauss syndrome), stool guiac, creatinine, LFT's, urine analysis, RF, ASO, ANA, ANCA, SPEP (Hypergammaglobulinemic purpura), Hepatitis B and C, ELISA for parvovirus B19, complement levels, cryoglobulins, anti C1Q antibody and IgA-fibronectin aggregates (if available) and CXR.

For the acutely ill patient with necrotic skin lesions, it is important to exclude septic emboli (e.g. Neisseria, Staph, RMSF, Strep, Pseudomonas, Candida). A skin biopsy with special stains for bacteria, fungi and AFB is in order. Sending another skin specimen for bacterial, fungal and AFB culture should also be done. One may aspirate tissue fluid from an early lesion for gram stain and culture.

Comments

PTU for thyroid disease can cause an ANCA-positive vasculitis.

Table of Laboratory and Diseases

Test Disease/Comments
Skin Biopsy Important for all
ANA Lupus Erythematosis
RF Rheumatoid Vasculitis
ANCA GPA (90%, usually cytoplasmic), CSS (40%)
Proteinase 3 and myeloperoxidase by ELISA Test if ANCA pos
Proteinaase 3 GPA
Myeloperoxidase Microscopic polyangiitis
Antiphospholipid Antibody Antiphopholipid Syndrome, and sometimes Livedo Vasculitis
Anticardiolipin Antibody Lupus Erythematosis
CBC with eosin count CSS
SPEP Hypergammaglobulinemic Purpura
Cryoglobulins Cryoglobulinemia
DIF (positive IgA) Henoch-Schonlein Purpura
Factor V Leiden heterozygous mutation Factor V Leiden heterozygous mutation
C3 and C4 levels Usually elevated. Low in hypocomplementemic urticarial vasculitis
CXR GPA, CSS, Microscopic polyangiitis, Takayasu artritis and Behcets Syndrome

GPA=granulomatosis with polyangiitis (formerly Wegeners), CSS=Churg-Strauss-Syndrome

Vasculitides without Specific Blood Markers

Polyarteritis Nodosa
Pyoderma Gangrenosum
Livedoid Vasculopathy

Thrombophilia Workup

Thrombophilia (sometimes hypercoagulability or a prothrombotic state) is an abnormality of blood coagulation that increases the risk of thrombosis (blood clots in blood vessels). Thrombophilia work-up includes protein C, protein S, antithrombin III, factor V Leiden mutation, homocysteine, prothrombin gene mutation (G20210A), and methylene tetrahydrofolate reductase (MTHFR) mutation.

Factor V Leiden Thrombophilia

Factor V Leiden thrombophilia is an inherited disorder of blood clotting. People with factor V Leiden thrombophilia have a higher isk of deep venous thrombosis, pulmonary embolus and in women, miscarriages.

See also livedo reticularis.

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