Further Outpatient Care

Close follow-up of patients with systemic vasculitis by a multidisciplinary team is extremely important for monitoring progression of disease, response to therapy, and complications.

Patients with Henoch-Schönlein purpura should have periodic checks of urinalysis as long as 6 months after the acute illness to screen for delayed-onset nephritis.^[26]

Patients with Kawasaki disease should have follow-up with echocardiogram at 6-8 weeks after acute illness. Most centers are also doing follow-up echocardiogram at 1 year.

Patients receiving cyclophosphamide should have close monitoring of CBC count for cytopenias.

Patients receiving rituximab should have regular monitoring of immunoglobulin levels and CD19⁺ lymphocyte count.

For patients with elevated antineutrophil cytoplasmic antibody (ANCA), titers may normalize during periods of disease control and increase with disease activity. Serial ANCA titers have been used to measure disease activity, with limited success. An acute rise in ANCA titer suggests clinical activity; persistent high titers are less helpful in identifying patients with active disease.

Transfer

Transfer to tertiary care center is indicated for patients with the following:

Kawasaki disease complicated by myocarditis, shocklike syndrome, and refractory disease
Henoch-Schönlein purpura complicated by significant renal impairment and CNS involvement
Patient who is suspected to have Takayasu arteritis (TA), polyarteritis nodosa, antineutrophil cytoplasmic antibody-associated vasculitis, Behçet disease, childhood primary angiitis of the CNS (PACNS)
Significant organ involvement or compromise (ie, renal, CNS, cardiac)

Deterrence/Prevention

Primary prevention of systemic vasculitis is not usually possible.

One must maintain a high index of suspicion for this group of diseases because early diagnosis and appropriate aggressive treatment is essential.

Prevention of renal disease in Henoch-Schönlein purpura has been an area of controversy in the literature. Treatment with corticosteroids may prevent progression of renal disease; however, a true benefit has not been proven.^[39]

Prophylaxis for P jiroveci with trimethoprim-sulfamethoxazole is indicated for patients being treated with cyclophosphamide.

All patients receiving prednisone should have monitoring of bone mineral density and should ensure good intake of calcium and vitamin D.

Secondary thromboprophylaxis indicated for patients with thrombotic event and hypercoagulable state/antiphospholipid antibody syndrome.

Questions

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Levine D, Akikusa J, Manson D, Silverman E, Schneider R. Chest CT findings in pediatric Wegener's granulomatosis. Pediatr Radiol. 2007 Jan. 37(1):57-62. [QxMD MEDLINE Link].
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Media Gallery

Preferred sites of vascular involvement by selected vasculitides.
Patient with Wegener granulomatosis and saddle-nose deformity.
Tender erythematous nodules in cutaneous polyarteritis nodosa (PAN).
Nodules on sole of foot in cutaneous polyarteritis nodosa (PAN).
Necrotic lesions of polyarteritis nodosa (PAN).
Chest radiography in Churg-Strauss syndrome (CSS) with pulmonary infiltrates.
CT of sinuses in a patient with Wegener granulomatosis (WG) showing erosion and loss of sinus walls.
CT chest in a patient with Churg-Strauss syndrome (CSS) showing multiple nodules.

of 8

Sections Vasculitis and Thrombophlebitis
Overview
Presentation
DDx
Workup
Treatment
Medication
Follow-up
Questions & Answers
Media Gallery
Tables
References

Category	Definition
Localized	Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms
Early systemic	Any, without organ-threatening or life-threatening disease
Generalized	Renal or other organ-threatening disease, serum creatinine >500 μmol/L (5.6 mg/dL)
Severe	Renal or other vital organ failure, serum creatinine >500 μmol/L (5.6 mg/dL)
Refractory	Progressive disease unresponsive to glucocorticoids and cyclophosphamide

Vasculitis and Thrombophlebitis Follow-up

Further Outpatient Care

Transfer

Deterrence/Prevention

References

Tables

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