Urticarial vasculitis: diagnosis, clinical presentation & treatment paradigm – a UCARE study: Pavel Kolkhir, EAACI 2023

Urticarial vasculitis is a rare inflammation of the blood vessels which results in clinical manifestations resembling urticaria with the addition of wheals, there remain no direct treatment options and management involves antihistamines, NSAIDS and ibuprofen to treat the cutaneous manifestations. We were delighted to speak to Dr. Pavel Kolkhir (Charité-Universitätsmedizin Berlin, Berlin, Germany) around the pathology and presentation of UV, the challenges in diagnosis and treatment, and the aims, methodology and findings from the UCARE study presented at EAACI.

The abstract ‘Urticarial vasculitis differs from chronic spontaneous urticaria in time to diagnosis, clinical presentation and need for anti-inflammatory treatment: An international prospective UCARE study.’ was presented at the European Association of Allergy & Immunology (EAACI) conference, 09-11 June 2023. Dr. Pavel Kolkhir was awarded a poster prize for outstanding poster presentation for this abstract.

Questions

1. Please could you describe the pathology, prevalence and clinical presentation of urticarial vasculitis (UV)? (0:46)
2. What challenges exist in the diagnosis and management of UV and what is the current standard of care? (1:48)
3. Please could you describe the aims and methodology of the UCARE study you presented at EAACI? (2:43)
4. What were the major findings of the study in terms of diagnosis, clinical presentation and treatment? (3:36)
5. How might this study help in distinguishing UV from CSU and emphasise the need for effective diagnostics, treatments and guidelines in UV? (4:41)

Disclosures: Pavel Kolkhir has nothing to disclose in relation to this video interview.

Support: Interview and filming supported by Touch Medical Media Ltd. Interview conducted by Victoria Jones.

Filmed in coverage of the EAACI Annual Meeting.

Click here for more content on urticaria & read more on the UCARE study here.

Transcript

Please could you describe the pathology, prevalence and clinical presentation of urticarial vasculitis (UV)? (00:47)

So urticarial vasculitis is a rare disease, so less than 10% of patients who initially present with chronic urticaria, they have urticarial vasculitis. So it is characterized by skin symptoms such as wheals, angioedema, burning on the skin, skin pain, skin and eye pigmentation, as well as non-skin related symptoms or systemic symptoms such as fever, joint pain, and eye inflammation. Pathophysiologically, urticarial vasculitis is a disease, which is characterized by type 3 hypersensitivity reaction according to the Gell and Coombs classification, with antigen-antibody complexes deposition in the vessel walls, and its complement consumption.

What challenges exist in the diagnosis and management of UV and what is the current standard of care? (01:48)

So it’s a challenge to differentiate between chronic spontaneous urticaria and urticarial vasculitis, and we can do this with the help of skin biopsy where we can see leukocytoclastic vasculitis, with leukocytoclasis, erythrocyte extravasation and fibrin deposition in the case of urticarial vasculitis, of course. Urticarial vasculitis is also a difficult-to-treat disease, and there is no safe and effective treatment, unfortunately and antihistamines, which are effective in the case of chronic spontaneous urticaria, are rarely effective in patients with urticarial vasculitis. Of course, we can use systemic corticosteroids, which can help these patients, but they have a broad range of side effects.

Please could you describe the aims and methodology of the UCARE study you presented at EAACI? (02:42)

Our study involved ten international urticaria centres from different countries, which recruited more than 100 patients with chronic spontaneous urticaria and the same number of patient with urticarial vasculitis. The main aim was to compare these two groups in terms of patient demographic characteristics, disease clinical characteristics and response to treatment, to find possible parameters which would help us in diagnosis and differential diagnosis of urticarial vasculitis and might be indication for performance in biopsy in patients who initially present with chronic urticaria.

What were the major findings of the study in terms of diagnosis, clinical presentation and treatment? (03:35)

So the major findings were that patients with urticarial vasculitis show high rates and also a high risk of having wheals of more than 24 hours in duration, skin hyperpigmentation, skin pain, and systemic symptoms such as eye inflammations, compared to patients with chronic spontaneous urticaria. Also, we can classify urticarial vasculitis on the basis of compliment consumption into normocomplementemic urticarial vasculitis and hypocomplementemic urticarial vasculitis, and patients with normocomplementemic urticarial vasculitis showed a longer diagnostic delay compared to patients with chronic spontaneous urticaria, and patients with hypocomplementemic urticarial vasculitis. Finally, patients with urticarial vasculitis have a high need for anti-inflammatory and immunosuppressive treatment as compared to patients with chronic spontaneous urticaria.

How might this study help in distinguishing UV from CSU and empathise the need for effective diagnostics, treatments and guidelines in UV? (04:42)

The criteria which were found in our study might help in differential diagnosis and diagnosis of urticarial vasculitis and patients who initially present with chronic urticaria. So we can, with a patient with chronic wheals, chronic hand wheals, shows wheals of more than 24 hours in duration, skin eye hyperpigmentation, skin pain, or any stomach symptoms, so one of these symptoms or several of them, then this might be a possible indication for performing a skin biopsy in this patient. And a second point is that, unfortunately, as I already said, many patients with urticarial vasculitis, they do not respond to a conventional treatment to antihistamines, and we need better therapies for this disorder.

Subtitles and transcript are autogenerated.