CLINICAL REPORT
641 ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Clinical Features of Chronic Spontaneous Urticaria that Predict Disease Prognosis and Refractoriness to Standard Treatment
Laia CURTO-BARREDO 1 , Laura RIBA ARCHILLA 2 , Guillem ROURA VIVES 2 , Ramon M . PUJOL 1 and Ana M . GIMÉNEZ-ARNAU 1
1
Department of Dermatology , Hospital del Mar – Institut Mar d ’ Investigacions , Mèdiques ( IMIM ), Universitat Autònoma de Barcelona ( UAB ), and 2 Adknoma Health Research , Barcelona , Spain
Chronic spontaneous urticaria ( CSU ) is characterized by heterogeneous activity , evolution , associated comorbidities and response to treatment . The aim of this study was to identify prognostic factors in patients with CSU that predict disease course and response to standard treatments . An observational retrospective study was conducted in a cohort of 549 patients with CSU , comparing patients with isolated CSU and those with CSU with concomitant inducible urticaria ( CSU- CIndU ). The factors associated with a worse prognosis in terms of duration and / or CSU activity and its episodes were : multiple episodes of CSU ( 19.2 % had more than one lifetime episode of CSU ), late-onset ( 63.6 % of patients developed first onset of CSU after the age of 45 years ), concomitant CIndU ( 20.2 %) and functional serum auto reactivity . Patients with CSU-CIndU required more frequent therapy after 5 years and higher doses of 2 nd -generation H1-antihistamines . Of patients with a baseline Urticaria Activity Score 7 ( UAS7 ) between 16 and 42 , 84.6 % required cyclosporine or omalizumab to achieve symptom control , compared with 15.4 % of patients with a baseline UAS7 between 0 and 15 ( p = 0.0013 ). Baseline CSU activity is the only factor found to be predictive for refractoriness to treat ment with H1-antihistamines .
Key words : chronic spontaneous urticaria ; disease activity ; prognosis ; serum autoreactivity ; antihistamine refractoriness .
Accepted Apr 12 , 2018 ; Epub ahead of print Apr 12 , 2018 Acta Derm Venereol 2018 ; 98 : 641 – 647 .
Corr : Ana M . Giménez-Arnau , Department of Dermatology , Hospital del Mar – Institut Mar d ’ Investigacions Mèdiques ( IMIM ), Universitat Autònoma de Barcelona ( UAB ), Passeig Marítim 25 – 29 , ES-08003 Barcelona , Spain . E-mail : anamariagimenezarnau @ gmail . com , 22505aga @ gmail . com
Chronic urticaria ( CU ) is a condition characterized by wheals , angioedema or both , lasting for more than 6 weeks , and sometimes persisting for years . Spontaneous CU ( CSU ) has unpredictable symptoms , while inducible CU ( CIndU ) is provoked by , for example , cold , heat , pressure , friction , or contact e . g with proteins , among other factors . Both types can be present concomitantly . The prevalence of CSU in the general population ranges between 0.5 % and 1 % ( 1 – 4 ). CSU has a significant negative impact on patients ’ quality of life , and is a considerable social and healthcare burden ( 5 – 7 ). Some clinical and laboratory parameters have been suggested to be predictive of more persistent and treatment-resistant
SIGNIFICANCE
• The activity and the clinical course of chronic spontaneous urticaria ( CSU ) differ widely between patients .
• Clinical predictors of longer course of CSU include lateonset ( 63.6 % showed > 45 years once the CSU started ), a concomitant chronic inducible urticaria ( CIndU ) and a relapsing course showing multiple episodes of CSU along the life . Higher CSU activity is shown with serum autoreactivity and concomitant CIndU .
• More than 75 % of patients were refractory to first-line treatment with licensed doses of H1-antihistamines . The baseline UAS7 has been demonstrated to be the unique parameter able to predict refractoriness to H1-antihistamines .
• Almost 90 % of patients with baseline UAS7 > 16 needed cyclosporin A or omalizumab combined with antihistamines in order to control CSU symptoms .
CSU ( 4 , 8 ). Autoimmunity may be involved in CSU with higher activity and longer duration ( 9 – 14 ). However , the significance of such observations is controversial ( 15 ).
The aim of this study was to identify specific demographic and phenotypic features in a large cohort of patients with CSU that may help to define prognostic factors , and predict disease course and the response to standard treatments , especially H1-antihistamines . The recognition of distinctive characteristics in the patients treated in our clinics would be of value in our daily practice .
MATERIALS AND METHODS Study design and patient population
This observational study retrospectively reviewed the medical records of 1,056 patients who were registered with the Urticaria Unit of the Hospital del Mar , Barcelona , Spain , from 2001 to 2014 . From a total of 997 patients aged over 16 years with a diagnosis of urticaria ( acute urticaria , CSU or CIndU ), data for 549 patients with CSU were included in the statistical analysis . Clinical features ( Table SI 1 ), laboratory parameters and extended complementary studies ( Table SII 1 ), prescribed drugs , response to treatment and evolution data were systematically entered in an electronic case-report form ( www . adknoma . com / URTICARIA / login . aspx ). CSU features involving activity , diagnostic tools , complementary exploratory tests , comorbidities and therapeutic recommendations were assessed according the EAACI / GA 2 LEN /
1 https :// www . medicaljournals . se / acta / content / abstract / 10.2340 / 00015555-2941
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2941 Acta Derm Venereol 2018 ; 98 : 641 – 647