Urticarial vasculitis (UV), a rare chronic skin disease characterized by urticarial lesions and leukocytoclastic vasculitis, can be difficult to treat due to a lack of clinical guidelines and the limited efficacy of current drug options. At the same time, there is little research on the patient experience throughout the clinical course of UV. A study accepted for publication in The Journal of Allergy and Clinical Immunology: In Practice, found that UV and its management can significantly impact quality of life (QOL).
UV typically manifests as long-lasting wheals leukocytoclastic vasculitis identified by histopathologic findings. Symptoms vary, from mild skin irritation to severe systemic disease. Burning and pain often occur with wheals, and hyperpigmentation is common. Many patients also experience angioedema, and the kidneys, lungs, eyes, nose, gastrointestinal system, and nervous system may be involved. The median age of onset is 45 years, and 70% to 74% of patients are female.
There is no approved treatment for UV. Although corticosteroids are effective, they carry significant adverse effects. UV is divided into subtypes based on complement consumption: normocomplementemic UV (NUV), characterized by normal levels of complement component 3 (C3) and C4, and hypocomplementemic UV (HUV), characterized by decreased C3 and/or C4 levels. NUV and HUV affect approximately 80% and 20% of patients, respectively.
The current study included 87 participants who filled out a Facebook survey for patients with UV. All patients included had wheals and biopsy-confirmed leukocytoclastic vasculitis. The survey aimed to clarify patient experiences and QOL related to clinical symptoms of UV, associated diseases, and the effects of currently available treatments.
Most of the survey participants were female (94.3%) and all had confirmed chronic UV, defined as lasting longer than 6 weeks. The mean patient age was 47 years, mean age at onset was 37 years, and the average diagnostic delay was 8.1 months. All patients had wheals, 92% had postinflammatory hyperpigmentation, and 91.9% had angioedema. Skin itching, burning, and pain affected 89.7%, 88.5%, and 81.6% of patients, respectively. Most patients (97.7%) also reported experiencing at least 1 systemic symptom in addition to skin symptoms. More patients with HUV or HUV syndrome (HUVS) reported lymph node swelling vs patients with NUV (69.6% vs 40.7%).
Most patients (51.8%) reported severely or very severely decreased QOL resulting from UV, and low QOL was the top reported greatest challenge related to UV (40.7% of patients). UV’s long duration, frequent relapses, and lack of a cure or confirmed cause were also significant burdens to patients. Those who reported severely decreased QOL had the highest median diagnostic delay.
Longer duration of disease and higher systemic symptom numbers correlated with lower QOL, although the correlation was not strong. Allergies, lung disease, and chronic infections were associated with more significant QoL decreases compared with patients who did not have these comorbidities. In patients with HUV or HUVS, lower QOL correlated with undergoing treatment with a higher number of drugs.
The study also found that UV symptoms are difficult to manage via therapy, with just 12.5% of patients on antihistamines, the most commonly used treatment (82.8%) showing significant improvement. A further 31.9% of patients on antihistamines reported no improvements or even worse symptoms. Hydroxychloroquine and methotrexate, both oral corticosteroids, were the most effective agents in this population. A total of 44.5% of patients were treated with oral corticosteroids, of whom 56.4% reported significant symptom improvement, 20.5% reported slight or moderate symptom improvement, and 7.7% had no change or worsened symptoms.
“To our knowledge, this is one of the first studies aimed exclusively at an existing Facebook support group with affected patients and the first study that involved UV patients and assessed their experience, needs, and preferences,” the authors wrote. “Our results based on patient-reported information demonstrate that UV patients have a considerable impairment of QOL associated with long disease duration, high symptom burden, and high need for therapy.” Early disease control may decrease the overall burden and increase QOL.
UV is rare and remains challenging to manage, but the findings suggest that patient QOL needs to be considered as disease management is planned. Further research using multidimensional validated tools is warranted to identify and address unmet needs for patients with UV, and targeted treatments must be developed to optimize outcomes, the authors concluded.