Q:

11/18/2013
Dermatology dept recently referred a 44 year-old female with 4 month history of urticaria. Patient was not responding to usual therapy for hives and subsequent biopsy was consistent with a diagnosis of leukocytoclastic vasculitis (the patient's hives were persisting for more than 48 hours and were bruising). Laboratory screen for vasculitis work-up was completely negative. Dermatologist is considering cyclosporine, but wonders if Xolair might be an option.

I have had success treating a few patients with CU with Xolair, but wondering if there's any reason not to consider a trial for this patient with urticarial vasculitis.

A:

Thank you for your inquiry.

You are correct in that omalizumab is an effective agent in chronic idiopathic urticaria. Cyclosporine is also effective in this disorder. There is a nice review of alternative treatments of urticaria by Dr. Allen Kaplan (see abstract copied below). In this article, Dr. Kaplan compares the two treatments.

Both omalizumab (1, 2) and cyclosporine have been used in treating forms of vasculitis. However, I am not aware of, nor could I find any reference to, the use of omalizumab per se in a patient with documented urticarial vasculitis. It has been used successfully in Churg-Strauss. It would not be my personal first choice to treat an urticarial vasculitis. I think cyclosporine would be more appropriate, but you might also consider the agents mentioned by Dr. Kaplan noted in the abstract copied below.

Thank you again for your inquiry and we hope this response is helpful to you.

References:
1) Omalizumab treatment associated with Churg-Strauss vasculitis.
Bargagli E, Rottoli P. Int Arch Allergy Immunol. 2008; 145(3):268. Epub 2007 Oct 5

2) Churg-Strauss vasculitis in a patient treated with omalizumab.
Bargagli E, Madioni C, Olivieri C, Penza F, Rottoli P. J Asthma. 2008 Mar; 45(2):115-6.

Allergy Asthma Immunol Res. 2012 Nov;4(6):326-31. doi: 10.4168/aair.2012.4.6.326. Epub 2012 May 14.
Treatment of chronic spontaneous urticaria.
Kaplan AP.
Source
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Allergy and Clinical Immunology, Medical University of South Carolina, Charleston, SC, USA.
Abstract
Chronic spontaneous urticaria is defined as persistent symptoms of urticaria for 6 weeks or more. It is associated with autoimmunity in approximately 45 percent of patients. Therapy is often difficult however the initial approach should employ high-dose non-sedating antihistamines; 4-6 tablets/day may be necessary. It has been shown that the response to 4 tablets/day exceeds 3, and exceeds 2, which exceeds 1. However the dose that corresponds to the maximal dose of first generation antihistamines (hydroxyzine, diphenhydramine) used previously, is 6/day. Yet over half the patients are refractory to antihistamines and other agents should be tried next. Whereas current guidelines (published) often add leukotriene antagonists and/or H(2) receptor antogonists next, these are of little utility. Likewise drugs effective for urticarial vasculitis (colchicine, dapsone, sulfasalazine, hydroxychloroquine) are effective in a small percentage of patients and no study suggests that the response rate of any of them exceeds the 30% placebo responses seen in most double-blind, placebo controlled studies. The drugs that are effective for antihistamine-resistant chronic spontaneous urticaria are corticosteroids, cyclosporine, and Omalizumab. Use of steroids is limited by toxicity. If used at all, a dose of no more than 10 mg/day should be employed with a weekly reduction of 1 mg. The response rates to cyclosporine and Omalizumab are each close to 75%. Cyclosporine can be used effectively if care is taken to monitor blood pressure, urine protein, blood urea nitrogen, and creatinine, every 6 weeks. Omalizumab has the best profile in terms of efficacy/toxicity and, once approved by federal agencies for use in chronic spontaneous urticaria, a dramatic change in the treatment paradigm, whether associated with autoimmunity or not, is predicted. A phase 3 trial is currently in place. Refractoriness to both Omalizumab and cyclosporine is expected to be less than 5 percent of patients. Other agents, can then be tried.

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology