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Vasculitis and Thrombophlebitis Follow-up

  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jun 30, 2015
 

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.[20]

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.[29]

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.[44, 45] 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.[46]

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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Contributor Information and Disclosures
Author

Nadia Jennifer Chiara Luca, MD Fellow in Pediatric Rheumatology, University of Toronto Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Susanne Maria Benseler, MD Pediatric Rheumatologist, Section Chief, Alberta Children's Hospital; Associate Professor, Department of Pediatrics, University of Calgary Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Barry L Myones, MD Co-Chair, Task Force on Pediatric Antiphospholipid Syndrome

Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Christine Hom, MD, to the development and writing of this article.

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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.
Table 1. EUVAS disease categorization of ANCA-associated vasculitis
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
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