RESUMO
BACKGROUND: Long-term, real-word data are needed to help manage patients with hidradenitis suppurativa (HS) through this recurrent, painful and debilitating disease. OBJECTIVES: To primarily measure real-world effectiveness of adalimumab in HS and to secondarily observe clinical course of HS in the light of patients' response. METHODS: In SOLACE, adults with moderate-to-severe HS in need for change in ongoing therapy were treated with adalimumab for up to 52 weeks as per physician's medical practice. Treatment effectiveness was measured by Hidradenitis Suppurativa Clinical Response (HiSCR). Inflammatory nodules, abscesses and draining fistulas were counted, Hurley stage was assessed, and disease severity was rated using the International HS Severity Scoring System (IHS4). A post hoc analysis further explored the HiSCR response by abscess and inflammatory nodule (AN) count at baseline (low, medium and high) and gender. Spontaneously reported safety events were collected. RESULTS: From 23 Canadian centres, 69% of the 138 patients achieved HiSCR at week 24, which increased to 82% and 75% at week 52 in patients with medium and high AN counts, respectively. Gender (4 times the odds for female) and age at HS onset (5% decrease with each additional year) had an effect on achieving HiSCR. Treatment with adalimumab led to an important decrease in number of lesions in responders, with most gains observed in inflammatory nodules, more frequently in the lower body area of patients in the high AN count group. The IHS4 scores of responders were substantially lowered, with a larger decrease in patients of the high AN count group. No new safety signal was detected. CONCLUSIONS: The effectiveness of adalimumab was maintained during this 1-year period, and an optimal gain was documented for patients with medium and high AN counts. These real-world data support a prompt treatment of HS patients and the use of IHS4 to monitor treatment.
Assuntos
Hidradenite Supurativa , Adalimumab/uso terapêutico , Canadá , Feminino , Hidradenite Supurativa/tratamento farmacológico , Humanos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
BACKGROUND: Immunotherapy with diphencyprone (diphenylcyclopropenone) is used in the treatment of alopecia areata (AA). Response rates have varied in the literature. OBJECTIVES: To determine the efficacy of diphencyprone therapy for AA in the largest reported cohort of patients; to identify patient and treatment factors predictive of therapeutic success; and to develop a practical model for predicting patient response. METHODS: The medical records of 148 consecutive patients treated with diphencyprone were reviewed. A clinically significant response to diphencyprone therapy was defined as a cosmetically acceptable response or greater than 75% terminal hair regrowth. Survival analyses using the Kaplan-Meier method and the Cox proportional hazards model were performed to determine significant factors predictive of regrowth and relapse. RESULTS: Using a survival analysis model, the cumulative patient response at 32 months was 77.9% (95% confidence interval, 56.8%-98.9%). Variables independently associated with clinically significant regrowth were age at onset of disease and baseline extent of AA. Older age at onset of AA portended a better prognosis. A cosmetically acceptable end point was obtained in 17.4% of patients with alopecia totalis/universalis, 60.3% with 75% to 99% AA, 88.1% with 50% to 74% AA, and 100% with 25% to 49% AA. A lag of 3 months was present between initiation of therapy and development of significant hair regrowth in the first responders. Relapse after achieving significant regrowth developed in 62.6% of patients. CONCLUSIONS: Response to diphencyprone treatment in AA is affected by baseline extent of AA and age at disease onset. A prolonged treatment course might be necessary. A predictive model has been developed to assist with patient prognostication and counseling.
Assuntos
Alopecia em Áreas/tratamento farmacológico , Ciclopropanos/uso terapêutico , Imunoterapia , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Teóricos , Estudos RetrospectivosRESUMO
BACKGROUND: Actinic prurigo (AP) is an idiopathic familial photodermatitis. AP of the Inuit is rarely reported and poorly characterized. OBJECTIVE: Our purpose was to examine the clinical features and HLA associations of AP in an Inuit population. METHODS: Thirty-seven Inuit subjects with AP were administered a questionnaire and underwent a cutaneous examination. Other causes of photosensitivity were excluded. HLA class I typing was performed by polymerase chain reaction and sequence-specific primers and class II typing by polymerase chain reaction and sequence-specific oligonucleotide probes. RESULTS: Subjects were 81.1% female, 67.6% had a family history of photosensitivity, and all experienced seasonal variation. The average age at onset of photosensitivity was 29 years, and only 27% had a trend toward improvement in photosensitivity. Involvement of eyes and nonexposed skin was reported in 62.2% and 18.9% of subjects, respectively. Physical examination revealed involvement of the face (64.9%), lip (32.4%), ear (13.5%), and dorsal aspect of the hand (24.3%). HLA-DRB1*14 was present in 51.2% of subjects and 26.2% of controls (P =.022, odds ratio = 2.975). This is a previously unreported HLA association. CONCLUSION: AP in the Inuit is a seasonal, pruritic photodermatitis, often commencing in adulthood and worsening over time. A novel association with HLA-DRB1*14 has been discovered. Overall, this novel HLA association, the absence of HLA associations previously reported in non-Inuit populations, and clinical distinguishing features support the concept that AP in the Inuit may have a distinct immunopathogenic basis that translates into a different phenotype. It also raises the question of whether AP in the Inuit is a distinct photosensitivity disorder specific to this group that has been genetically isolated because of geographic and cultural seclusion.