Urticarial vasculitis associated with low patient-reported quality of life

The long disease duration, high symptom burden and great need for therapy associated with urticarial vasculitis negatively impacted patient quality of life, according to study results.

Despite these challenges, current treatment is limited and no clinical guidelines are available, indicating a need for more research, Hanna Bonnekoh, MD, of the Institute of Allergology at Charité-Universitätsmedizin Berlin, and colleagues wrote in the study, published in The Journal of Allergy and Clinical Immunology: In Practice.

Characterized by wheals lasting longer than 24 hours and leukocytoclastic vasculitis, urticarial vasculitis (UV) impacts approximately 0.5 patients per 100,000 person-years in the United States.

Between 70% and 74% of patients are female, with a median age at onset of 45 years and median disease duration of 1.5 to 4 years. Presentations range from mild skin symptoms to severe systemic disease, with most cases having no underlying cause identified.

No treatment has been approved, and no treatment algorithms are available. Cutaneous and systemic symptoms have been treated with corticosteroids, but with considerable side effects, according to the researchers.

Between August 2020 and November 2020, the researchers disseminated an online survey that included 22 questions via a Facebook group of patients with UV, with 87 respondents (94.3% women; mean age, 47.3 ± 12.3 years; mean age at onset, 37.2 ± 12.9 years). All the patients had chronic UV with durations longer than 6 weeks and an average diagnostic delay of 8.1 months (interquartile range, 2-46).

The cohort consisted of 54% patients with normocomplementemic UV (NUV) and 46% with hypocomplementemic UV (HUV) or HUV syndrome (HUVS).

Symptoms included wheals (100%), postinflammatory hyperpigmentation (92%), angioedema (91.9%), itching (89.7%), burning of the skin (88.5%) and pain of the skin (81.6%), with 2.3% only reporting skin symptoms.

Also, 97.7% reported at least one systemic symptom such as lethargy or fatigue (86.2%), joint swelling or pain (75.9%), and muscle or bone aches (74.7%).

Patients with HUV or HUVS reported lymph node swelling more often than patients with NUV (696% vs. 40.7%; P = .042) as well as a higher mean number of systemic symptoms (12.4 vs. 11.3; P = .043).

Additionally, 51.8% reported severely or very severely decreased quality of life (QoL) due to the disease, with 3.6% reporting no change. These patients experienced the highest median diagnostic delays as well (P = .056).

When asked to report their greatest challenges, the respondents reported low QoL (40.7%), long duration with frequent relapses (14.8%), the incurability of UV (6.2%), the unknown cause of the disease (6.2%) and severe itch (6.2%).

The researchers found a weak correlation between lower QoL and longer disease duration (r = .298; P = .02), more systemic symptoms (r = .239; P = .03) and number of all clinical symptoms (r = .294; P = .007).

Specifically, patients with NUV had an association between lower QoL and younger age (r = -.461; P = .018) and a higher number of all clinical symptoms (r = .395; P = .046).

Patients with UV also reported allergies and intolerances (45.9%), autoimmune and rheumatic diseases (36.8%), mental diseases (32.2%), lung diseases (25.3%) and chronic infections (18.4%).

Compared with respondents without these concomitant diseases, severe or very severe decreases in QoL were more common among patients with allergies and intolerances (74.4% vs. 31.8%; P < .001), lung diseases (72.7% vs. 44.3%; P = .022) and chronic infections (81.3% vs. 44.8%; P = .009).

For treatment, respondents received antihistamines (82.8%), hydroxychloroquine (54%), analgesics (52.9%), oral corticosteroids (44.8%) and dapsone (35.6%), with topical corticosteroids reported by 42.5%. However, only 12.5% of the patients on antihistamines saw significant improvement, with 31.9% not improving at all or seeing their symptoms get worse.

Also, 56.4% of patients on oral corticosteroids reported significant improvements, with 20.5% seeing slight or moderate improvement and 7.7% seeing no change or worse symptoms.

After oral corticosteroids, hydroxychloroquine and methotrexate appeared most effective, whereas dapsone, montelukast and colchicine were least effective in treating clinical symptoms.

The researchers further found a correlation between low QoL and a high number of drugs used (r = .435; P < .001), with this correlation appearing among patients with HUV and HUVS (r = .558; P = .009) but not those with NUV (r = .174; P = 394).

Compared with respondents with higher QoL, those with lower QoL were treated with analgesics (P = .022), dapsone (P = .007), montelukast (P = .002), omalizumab (P = .007) and colchicine (P = .045) more often.

Considering how UV considerably impairs QoL, the researchers called for studies assessing disease activity and QoL with appropriate tools as well as investigations into diagnostic delay and factors contributing to longer disease courses.

Also, the researchers wrote the higher need for therapy connected with low QoL should be addressed and that early symptom control could help decrease disease burden and improve QoL as well.