Genital ulcer severity score and genital health quality of life in Behçet’s disease

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Abstract

Background

Behçet’s Disease (BD) is a chronic auto-inflammatory, multisystem relapsing/remitting disorder of unknown aetiology. Oro-genital ulceration is a key feature of the disease and has a major impact on the patients’ quality of life. Other clinical manifestations include ocular inflammation, rheumatologic and skin involvement, while CNS and vascular complications can lead to considerable morbidity. The availability of a valid monitoring tool for BD activity is crucial in evaluating the impact of the disease on daily life activity. The aims of this study were to validate a novel tool for monitoring genital ulceration severity in BD and to assess the impact of genital ulcers on the Genital Health Quality of Life (GHQoL).

Methods

Genital Ulcer Severity Score (GUSS) was developed using six genital ulcer characteristics: number, size, duration, ulcer-free period, pain and site. A total of 207 BD patients were examined, (137 females: mean age ± SD: 39.83 ± 13.42 and 70 males: mean age ± SD: 39.98 ± 11.95) from the multidisciplinary Behçet’s Centre of Excellence at Barts Health NHS Trust. GUSS was used in conjunction with Behçet’s Disease Current Activity Form (BDCAF).

Results

The over-all score of GUSS showed a strong correlation with all genital ulcer characteristics, and the strongest correlation was with the pain domain (r = 0.936; P < 0.0001). Ulcer average size and ulcer pain were the major predicting factors in GUSS (β = 0.284; β = 0.275) respectively, and P-values were significant. Multivariate regression analysis indicated that the ulcer pain, size and site are the main ulcer characteristics having an influence on the GHQoL (R ²: 0.600; P < 0.0001).

Conclusions

This study established the practicality of GUSS as a severity monitoring tool for BD genital ulcers and validated its use in 207 patients. Genital ulcers of BD have a considerable impact on the patients GHQoL.

Electronic supplementary material

The online version of this article (doi:10.1186/s13023-015-0341-7) contains supplementary material, which is available to authorized users.

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Most cited references 36

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Behcet's disease: from East to West.

Cheyda Chams-Davatchi, Farhad Shahram, Farima Ashofteh … (2010)

Behcet's disease (BD) is classified among vasculitides. There are actually five nationwide surveys of BD: Iran, Japan, China, Korea, and Germany. Among case series, four are on more than 200 cases (Turkey, Morocco, Tunisia, and UK). BD was classically seen around the Silk Route. Now, it is seen everywhere. The male to female ratios were, respectively (in nationwide surveys), 1.19, 0.98, 1.34, 0.63, and 1.40 to 1. The mean age at onset was 26.2, 35.7, 33.8, 29, and 26 years. Major manifestations were seen, respectively, in nationwide surveys: mucous membrane (oral aphthosis in 97%, 98%, 98%, 99%, and 98%; genital aphthosis in 65%, 73%, 76%, 83%, and 64%); skin manifestations (pseudo-folliculitis in 57%, N/A, 31%, N/A, and 62%; erythema nodosum in 22%, N/A, 38%, N/A, and 42%; ocular manifestations in 55%, 69%, 35%, 51%, and 53%). Minor manifestations were seen, respectively, in nationwide surveys: joint manifestations in 33%, 57%, 30%, 38%, and 53%; neurological manifestations in 9%, 11%, 6.5%, 4.6%, and 11%; gastrointestinal manifestations in 7%, 15.5%, 9%, 7.3%, and 12%; vascular involvement in 8.9%, 8.9%, 7.7%, 1.8%, and 13%; pulmonary manifestations in 0.3%, N/A, 2.2%, N/A, and 3.6%; cardiac manifestations in 0.5%, N/A, 4%, N/A, and 3.2%. Laboratory tests are not useful except the pathergy test, which was positive in 54%, 44%, N/A, 40%, and 34% of cases. ESR was normal in many patients. Diagnosis is based upon clinical manifestations. The International Criteria for Behcet's Disease (ICBD, 2006) may be of help, having a sensitivity of 98.2% and a specificity of 95.6% in Iranian patients.

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Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease.

Umit Tursen, A Boyvat, A Gürler (2003)

Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients. To determine the influence of sex on the clinical course of Behçet' s disease. We retrospectively evaluated the clinical findings of 2313 Behçet patients followed up at the multidisciplinary Behçet's Disease Center at Ankara University. The male/female patient ratio was 1.03. Oral aphthae was seen in all patients. In male Behçet patients, the prevalence of mucocutaneous lesions and systemic manifestations was as follows: 85.6% genital aphthae, 45.5% erythema nodosum, 59.5% papulopustular lesions, 17.5% thrombophlebitis, 38.1% ocular involvement, 11.3% articular involvement, 11.7% vascular involvement, 3.3% neurologic involvement, 1.4% gastrointestinal involvement, and 1.8% pulmonary involvement. In female Behçet patients, the prevalence of manifestations were as follows: 91% genital aphthae, 49.8% erythema nodosum, 48.3% papulopustular lesions, 3.5% thrombophlebitis, 19.8% ocular involvement, 11.8% articular involvement, 2.1% vascular involvement, 1.3% neurologic involvement, 1.4% gastrointestinal involvement, and 0.03% pulmonary involvement. Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.

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Clinical features and natural course of Behçet's disease in 661 cases: a multicentre study.

L Donmez, S Buyukkara, Steven A. Akman … (2007)

Behçet's disease (BD) is a systemic inflammatory disease with unpredictable exacerbations and remissions. The natural course of BD is not fully known. We aimed retrospectively to determine the occurrence of the symptoms in chronological order. We also evaluated the influence of the treatment and follow-up on the clinical severity and tried to identify the factors determining severe organ involvement. Six hundred and sixty-one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient. Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85.3%), papulopustular lesions (55.4%), erythema nodosum (44.2%), skin pathergy reaction (37.8%), and articular (33.4%) and ocular involvement (29.2%). Oral ulcers were the most common onset manifestation (88.7%). The mean +/- SD duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4.3 +/- 5.7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0.001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy reaction were found to be significantly more frequent in males. Mucocutaneous lesions are the hallmarks of the disease, and especially oral ulcers precede other manifestations. The increase in clinical severity score is more pronounced in patients without regular treatment and follow-up. Male sex and a younger age at onset are associated with more severe disease.

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Author and article information

Contributors

Amal Senusi: a.a.s.senusi@qmul.ac.uk

Noha Seoudi: n.seoudi@qmul.ac.uk

Lesley Ann Bergmeier: l.a.bergmeier@qmul.ac.uk

Farida Fortune: +44(0)20 7882 8484 , f.fortune@qmul.ac.uk

Journal

Journal ID (nlm-ta): Orphanet J Rare Dis

Journal ID (iso-abbrev): Orphanet J Rare Dis

Title: Orphanet Journal of Rare Diseases

Publisher: BioMed Central (London )

ISSN (Electronic): 1750-1172

Publication date (Electronic): 22 September 2015

Publication date PMC-release: 22 September 2015

Publication date Collection: 2015

Volume: 10

Electronic Location Identifier: 117

Affiliations

[ ]Centre for Clinical and Diagnostic Oral Sciences, Institute of Dentistry, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK

[ ]Centre for Clinical and Diagnostic Oral Sciences, Institute of Dentistry, Barts and the London School of Medicine and Dentistry, Blizard Institute, 4 Newark Street, London, E1 2AT UK

Article

Publisher ID: 341

DOI: 10.1186/s13023-015-0341-7

PMC ID: 4579635

PubMed ID: 25603901

SO-VID: 68f03ff5-54f9-494b-ab05-dea3b7b8f5a3

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 25 June 2015

Date accepted : 8 September 2015

Custom metadata

ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: behçet’s disease,genital ulcer,severity,genital health quality of life

Data availability:

ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: behçet’s disease, genital ulcer, severity, genital health quality of life

Genital ulcer severity score and genital health quality of life in Behçet’s disease

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Most cited references 36

Behcet's disease: from East to West.

Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease.

Clinical features and natural course of Behçet's disease in 661 cases: a multicentre study.

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