Vasculitis and Thrombophlebitis Follow-up

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Follow-up

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. [21]

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.

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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)

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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. [33]

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.

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Complications

See the list below:

  • Destruction of paranasal sinuses

  • Subglottic stenosis requiring tracheostomy

  • Life-threatening pulmonary hemorrhage

  • Renal insufficiency requiring dialysis or transplant

  • Digital gangrene with autoamputation

  • Stroke

  • Myocardial infarction

  • Sepsis

  • Morbidity associated with immunosuppressive medications

  • Death

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Prognosis

Prognosis is related to the degree of end-organ involvement. Generally, ANCA-associated vasculitis is associated with a poorer prognosis

Recurrence rate in Kawasaki disease is approximately 2%. Patients with Kawasaki disease and large coronary aneurysms are at risk for multiple complications, including stenosis and obstruction, myocardial infarction, and dysrhythmias. Some experience with bypass grafting for revascularization has been reported very good success. [49, 50] In 2014, the largest US study of longer-term cardiac outcomes after Kawasaki disease reported a low rate of adverse cardiovascular events through age 21 years. [51]

The recurrence rate in Henoch-Schönlein purpura is approximately 30%.

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Patient Education

Patients receiving corticosteroids should be advised of possible side effects, including weight gain, sleep disturbance, hirsutism, glucose intolerance, and hypertension.

Patients receiving immunosuppressive agents should be instructed to seek medical attention with any sign of infection.

Patients taking methotrexate should avoid alcohol and other hepatotoxic substances.

Discussion of adequate birth control measures with patients of childbearing age is necessary if they are treated with teratogenic medications (eg, methotrexate, warfarin).

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