Evidence map›Paper›PMID 40455313›Full record

ReviewClinical rheumatology2025

Treatment and long-term follow-up of pediatric patients with hypocomplementemic urticarial vasculitis syndrome (HUVS): a case-based review.

Özen Taş, Fatma Aydın, Zeynep Birsin Özçakar

Abstract readCase ReportsReview
In one paragraph

Review in Clinical rheumatology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors.

Özen TaşDivision of Pediatric Rheumatology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.ORCID http://orcid.org/0000-0002-1132-8082
Fatma AydınDivision of Pediatric Rheumatology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.ORCID http://orcid.org/0000-0003-0306-7473
Zeynep Birsin ÖzçakarDivision of Pediatric Rheumatology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey. zbozcakar@yahoo.com.ORCID http://orcid.org/0000-0002-6376-9189

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare, severe form of urticarial vasculitis. It is characterized by persistent hypocomplementemia, chronic urticarial vasculitic lesions, and severe multiorgan involvement. Herein, we present long-term follow-up of two siblings diagnosed with HUVS at early ages, who were found to have DNASE1L3 mutations, and their subsequent 20-year follow-up. While one of the siblings developed lupus nephritis, the other exhibited vasculitic renal involvement. The patients received various treatments, with rituximab proving most effective in the long term. The present study contributes to the existing body of literature on pediatric HUVS, which, to the best of our knowledge, has been described in 28 cases. Renal involvement was present in 82% of patients, and lupus nephritis was most common in patients with renal pathology. Patients received many different treatments. Two patients died, and five patients developed end-stage renal failure. However, it should be noted that follow-up was not conducted in 39% of these patients and the follow-up period was very short for the remaining patients.

Indexed as

Complement System ProteinsRituximabUrticariaVasculitisAdolescentChildFemaleFollow-Up StudiesHumansLupus NephritisMaleMutationSyndromeComplement System ProteinsRituximabDNASE1L3 deficiencyHypocomplementemic urticarial vasculitisRenal involvement

Identifiers

PMID40455313
PMCPMC12234586

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.