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Indian Dermatology Online Journal 2024Alopecia areata (AA) presents with noncicatricial alopecia and has multifactorial etiology. Janus Kinase inhibitors (JAKibs) with potential efficacy and favorable...
A Real-World Study of Steroid-Free Monotherapy with Tofacitinib in Severe and Therapy-Recalcitrant Alopecia Areata, Alopecia Totalis, and Alopecia Universalis Cases: A Retrospective Analysis.
BACKGROUND
Alopecia areata (AA) presents with noncicatricial alopecia and has multifactorial etiology. Janus Kinase inhibitors (JAKibs) with potential efficacy and favorable side-effect profile are the first class of drugs to receive FDA approval in AA.
OBJECTIVES
Our primary objective was to assess the complete response rates to tofacitinib monotherapy in severe and recalcitrant AA, alopecia totalis (AT), and alopecia universalis (AU) patients using the latest percentage change in Severity of alopecia tool (SALT) score. We also aimed to analyze the various systemic agents used by these patients prior to the use of tofacitinib.
MATERIALS AND METHODS
Institutional records of 17 patients with severe or refractory AA, AT, and AU treated with tofacitinib monotherapy were analyzed, retrospectively. The response to tofacitinib therapy was determined after calculating percentage change in SALT score. End of treatment was defined as the dose which resulted in a significant response (complete/near complete response was ≥75% hair regrowth from baseline as determined by SALT score).
RESULTS
Majority of patients had severe AA (SALT ≥ 50) ( = 9/17, 52.94%), while five patients had AT and three had AU. All patients had received either systemic glucocorticoids (GCS), which included oral mini pulse (OMP) ( = 8), intravenous pulse steroids ( = 4), and daily oral GCS ( = 6) or immunosuppressive agents (ISAs) which included cyclosporine ( = 14) followed by methotrexate ( = 6) and azathioprine ( = 6). Mean SALT score prior to starting tofacitinib was 74.23. Mean dose of tofacitinib used was 13.23 mg (10-15 mg) and mean duration of treatment was 9.23 months. Latest percentage change of SALT score ranged from 70.58% to 100%, with an average of 91.47%. Most patients showed complete/near complete response (13/17, 76.47%).
CONCLUSION
Tofacitinib was found to be safe and effective in severe/refractory AA, AU, and AT patients recalcitrant to other treatment modalities in our study. Further studies are needed to assess the effect of these targeted drugs on JAK-STAT expression or tissue cytokines involved in the pathogenesis of AA using immunohistochemistry.
PubMed: 38282998
DOI: 10.4103/idoj.idoj_131_23 -
International Journal of Trichology 2018Alopecia areata (AA) is a T-lymphocyte-mediated disease that results in alopecia plaques or diffuses alopecia on the scalp and body. Etiologic factors include genetic...
BACKGROUND
Alopecia areata (AA) is a T-lymphocyte-mediated disease that results in alopecia plaques or diffuses alopecia on the scalp and body. Etiologic factors include genetic and autoimmune susceptibility. Treatment modalities are usually considered according to the extent of hair loss and the patient's age. Since there is no approved treatment by the US Food and Drug Administration, treatment options and combinations available are off-label. Patients with extensive AA (including totalis and universalis) have a low rate of spontaneous remission and poor treatment response. Extensive AA is usually associated with severe emotional distress, social discomfort, bullying, and other psychological problems for the child and family. In this context, the need for new therapeutic schemes is clear.
MATERIALS AND METHODS
We retrospectively analyzed five patients (aged 2-17 years) with extensive and refractory AA who were treated with mesalazine associated or not with oral prednisolone and topical betamethasone/minoxidil.
RESULTS
We observed complete growth of terminal hair in all patients. No patient had abnormal laboratory results or manifested drug side effects.
CONCLUSIONS
In extensive and refractory AA cases, the topical treatment combined with mesalazine may provide excellent results, reducing the need for extended oral corticosteroids courses. Besides that, mesalazine seems to minimize relapses on discontinuation of oral steroids. Controlled studies are needed to confirm the effectiveness of this combination.
PubMed: 30034187
DOI: 10.4103/ijt.ijt_14_18 -
Pediatric Rheumatology Online Journal Jan 2024STING-associated vasculopathy with onset in infancy (SAVI) is a rare type I interferonopathy caused by heterozygous variants in the STING gene. In SAVI, STING variants...
BACKGROUND
STING-associated vasculopathy with onset in infancy (SAVI) is a rare type I interferonopathy caused by heterozygous variants in the STING gene. In SAVI, STING variants confer a gain-of-function which causes overactivation of type I interferon (IFN) signaling leading to autoinflammation and various degrees of immunodeficiency and autoimmunity.
CASE PRESENTATION
We report the case of a 5 year old child and his mother, both of whom presented with systemic inflammatory symptoms yet widely varying organ involvement, disease course and therapeutic response. Genetic testing revealed a heterozygous STING variant, R281Q, in the child and his mother that had previously been associated with SAVI. However, in contrast to previously reported SAVI cases due to the R281Q variant, our patients showed an atypical course of disease with alopecia totalis in the child and a complete lack of lung involvement in the mother.
CONCLUSIONS
Our findings demonstrate the phenotypic breadth of clinical SAVI manifestations. Given the therapeutic benefit of treatment with JAK inhibitors, early genetic testing for SAVI should be considered in patients with unclear systemic inflammation involving cutaneous, pulmonary, or musculoskeletal symptoms, and signs of immunodeficiency and autoimmunity.
Topics: Child, Preschool; Humans; Immunologic Deficiency Syndromes; Inflammation; Interferon Type I; Lung; Mutation; Vascular Diseases; Male; Female
PubMed: 38178067
DOI: 10.1186/s12969-023-00934-4 -
Health Psychology Report 2024Alopecia is an autoimmune condition that results in hair loss, mainly from the scalp. There are three specific types of autoimmune alopecia: alopecia areata (AA; small...
BACKGROUND
Alopecia is an autoimmune condition that results in hair loss, mainly from the scalp. There are three specific types of autoimmune alopecia: alopecia areata (AA; small patches of hair loss), alopecia totalis (AT; total hair loss from the scalp) and alopecia universalis (AU; total hair loss from the scalp and body). Whilst research has explored the experiences of White women living with alopecia, there is a lack of research exploring the impact of alopecia on women in the Black community. The current study aimed to explore Black women's experience of living with autoimmune types of alopecia with a focus on the cultural importance of hair within the Black community and the impact of social support.
PARTICIPANTS AND PROCEDURE
Seven Black women (age range: 37-68 years; mean age: 51 years) were recruited purposively through alopecia support group organisations and social media to participate in a semi-structured interview; four participants were diagnosed with AA, two participants were diagnosed with AU, and one participant was diagnosed with AT. One-to-one interviews were conducted online, and interpretative phenomenological analysis was used to guide data collection and analysis.
RESULTS
Participants discussed the significance of hair specifically within the Black community and the complex relationship between psychological wellbeing, coping and seeking support.
CONCLUSIONS
This novel area, specific to Black women's psychological experience of alopecia, acknowledges the influence of cultural and ethnic differences. The findings suggest that proactive awareness from health professionals and social support groups are needed due to the nuances of Black women's alopecia experience to provide better support and to enhance the quality of life for Black women to manage their alopecia.
PubMed: 38628276
DOI: 10.5114/hpr/177730 -
Indian Journal of Dermatology,... 2015Severe, extensive, therapy resistant alopecia areata represents a clinical challenge. Systemic corticosteroids are a therapeutic tool that still needs to be evaluated. (Clinical Trial)
Clinical Trial
BACKGROUND
Severe, extensive, therapy resistant alopecia areata represents a clinical challenge. Systemic corticosteroids are a therapeutic tool that still needs to be evaluated.
AIM
The purpose of this study was to assess the efficacy and safety of methylprednisolone pulse therapy in alopecia areata and to find prognostic factors for a favourable outcome.
METHODS
A total of 32 patients with severe multifocal alopecia areata (more than 40% scalp hair loss), alopecia totalis, and alopecia universalis were treated with infusions of 500 mg methylprednisolone for 3 days every month for 3 consecutive months. The end point of the study was 12 months.
RESULTS
Of 32 patients, 26 (81.3%) reported a clinical response. Four patients (12.5%) showed complete hair regrowth, 6 patients (18.8%) showed >50% hair regrowth, ten (31.3%) had <50% hair regrowth, 6 (18.75%) were non responders, and another 6 patients (18.8%) had relapse after an initial regrowth. Multivariate analysis revealed that patients reporting at the first episode and those with multifocal disease had the best results.
CONCLUSION
Methylprednisolone infusions represent a possible therapeutic option for patients with multifocal alopecia areata and those presenting with the first episode of the disease.
Topics: Adolescent; Adult; Alopecia; Alopecia Areata; Anti-Inflammatory Agents; Child; Child, Preschool; Female; Humans; Infusions, Intravenous; Male; Methylprednisolone; Middle Aged; Prospective Studies; Recurrence; Severity of Illness Index; Time Factors; Treatment Outcome; Young Adult
PubMed: 25566921
DOI: 10.4103/0378-6323.148608 -
Anais Brasileiros de Dermatologia 2016Vitiligo is an acquired pigmentary skin disorder affecting 0.1-4% of the general population. The nails may be affected in patients with an autoimmune disease such as...
BACKGROUND
Vitiligo is an acquired pigmentary skin disorder affecting 0.1-4% of the general population. The nails may be affected in patients with an autoimmune disease such as psoriasis, and in those with alopecia areata. It has been suggested that nail abnormalities should be apparent in vitiligo patients.
OBJECTIVE
We sought to document the frequency and clinical presentation of nail abnormalities in vitiligo patients compared to healthy volunteers. We also examined the correlations between nail abnormalities and various clinical parameters.
METHODS
This study included 100 vitiligo patients and 100 healthy subjects. Full medical histories were collected from the subjects, who underwent thorough general and nail examinations. All nail changes were noted. In the event of clinical suspicion of a fungal infection, additional mycological investigations were performed.
RESULTS
Nail abnormalities were more prevalent in the patients (78%) than in the controls (55%) (p=0.001). Longitudinal ridging was the most common finding (42%), followed by (in descending order): leukonychia, an absent lunula, onycholysis, nail bed pallor, onychomycosis, splinter hemorrhage and nail plate thinning. The frequency of longitudinal ridging was significantly higher in patients than in controls (p<0.001).
CONCLUSIONS
Nail abnormalities were more prevalent in vitiligo patients than in controls. Systematic examination of the nails in such patients is useful because nail abnormalities are frequent. However, the causes of such abnormalities require further study. Longitudinal ridging and leukonychia were the most common abnormalities observed in this study.
Topics: Adolescent; Adult; Aged; Case-Control Studies; Child; Child, Preschool; Female; Humans; Hypopigmentation; Male; Middle Aged; Nail Diseases; Nails, Malformed; Prevalence; Statistics, Nonparametric; Turkey; Vitiligo; Young Adult
PubMed: 27579738
DOI: 10.1590/abd1806-4841.20164620 -
JAMA Dermatology Dec 2017The incidence of thyroid disease in children with alopecia areata (AA) has been widely studied with no consensus on whether a true association with AA exists. In...
IMPORTANCE
The incidence of thyroid disease in children with alopecia areata (AA) has been widely studied with no consensus on whether a true association with AA exists. In addition, screening practices for thyroid dysfunction in children with AA vary widely among clinicians.
OBJECTIVE
To reduce health care costs, eliminate unnecessary testing, and standardize clinical practices, we sought to characterize thyroid function in children with AA to establish guidelines for screening.
DESIGN, SETTING, AND PARTICIPANTS
A single-site retrospective medical chart review was carried out in an outpatient pediatric dermatology clinic in a tertiary referral medical center between January 1, 2008 and January 1, 2016. The study included 298 patients (ages 0-21 years) who received a clinical diagnosis of AA and underwent thyroid function tests.
MAIN OUTCOMES AND MEASURES
Age at diagnosis of AA, duration of disease, severity, location, and type were documented. Past medical history and family medical history of patients were also recorded. Results of laboratory tests including thyrotropin (formerly thyroid-stimulating hormone [TSH]), free T4 (FT4), triiodothyronine (T3), thyroid peroxidase antibodies (TPO-Abs), and thyroglobulin antibodies (Tg-Abs).
RESULTS
During the 8-year period, 298 patients with AA had thyroid function screening. Of those with thyroid screening, patterns of AA included patchy (68%), ophiasis (13%), totalis (9%), and universalis (10%). Severity was determined by percentage of hair loss on the scalp and were divided into mild (30.2%), moderate (32.9%), and severe (36.9%). A total of 59 (20%) patients had abnormalities on thyroid testing results. In this group of patients, hypothyroidism was the most frequent finding 29 (49%), with Hashimoto thyroiditis being the most common cause(24 [41%]). Other abnormalities included hyperthyroidism secondary to Grave disease (12 [20%]) and subclinical thyroid dysfunction (7 [12%]). Whereas age, duration of disease, pattern of alopecia, and diagnosis of autoimmune diseases had no significant association with abnormal thyroid findings, a personal history of Down syndrome (P = .004), atopy (P = .009), and family history of thyroid disease (P = .001) did.
CONCLUSIONS AND RELEVANCE
We recommend that routine thyroid function screening should be restricted to AA patients with a medical history of Down syndrome, personal history of atopy, a family history of thyroid disease, or clinical findings (goiter) suggestive of potential thyroid dysfunction in the individual patient.
Topics: Adolescent; Alopecia Areata; Autoimmune Diseases; Child; Child, Preschool; Female; Humans; Incidence; Infant; Male; Mass Screening; Practice Guidelines as Topic; Retrospective Studies; Severity of Illness Index; Thyroid Diseases; Thyroid Function Tests; Young Adult
PubMed: 28973128
DOI: 10.1001/jamadermatol.2017.3694 -
Skin Appendage Disorders Mar 2022Diphenylcyclopropenone (DPCP) is the medication of choice for the treatment of severe alopecia areata (AA) according to AA treatment guidelines. Precise initiation and...
INTRODUCTION
Diphenylcyclopropenone (DPCP) is the medication of choice for the treatment of severe alopecia areata (AA) according to AA treatment guidelines. Precise initiation and application are important factors for successful treatment. However, it is difficult for patients who live far away to visit their doctor weekly.
METHODS
We conducted a retrospective cohort study to assess the cost, effectiveness, and side effects of DPCP treatment between office-use DPCP (O-DPCP) and home-use DPCP (H-DPCP) in severe AA patients. A cost-effectiveness analysis was performed from the perspective of healthcare providers and patients using real-world data and the national cost statistics for hospital services comparing O-DPCP and H-DPCP in patients with severe AA at 24, 36, and 48 weeks.
RESULTS
Two groups of 41 patients treated with O-DPCP and H-DPCP were enrolled. There was no significant difference in the proportion of patients who showed a favorable outcome (≥50% improvement) with minimal side effects between both groups at 24 (O-DPCP 43.9% vs. H-DPCP 26.8%, = 0.11), 36 (O-DPCP 58.5% vs. H-DPCP 43.9%, = 0.19), or 48 weeks (O-DPCP 63.4% vs. H-DPCP 56.1%, = 0.49). The cost of H-DPCP was half of the cost of O-DPCP.
DISCUSSION/CONCLUSION
H-DPCP is a cost-effective and time-efficient alternative treatment option for severe AA patients.
PubMed: 35419425
DOI: 10.1159/000520568 -
Saudi Medical Journal Jun 2016To assess the significance of thyroid autoimmune screening in alopecia areata (AA) patients in Saudi population, and to determine whether there is a difference in...
OBJECTIVES
To assess the significance of thyroid autoimmune screening in alopecia areata (AA) patients in Saudi population, and to determine whether there is a difference in thyroid autoimmune susceptibility between mild and severe AA.
METHODS
In a prospective case-control study, we included 50 alopecia totalis (AT) and alopecia universalis (AU) patients, 50 age- and gender-matched patients with localized AA, and 50 age- and gender- matched healthy subjects between March 2015 and August 2015. Patients with AA were consecutively recruited from the hair disorders out-patient clinic of King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia.
RESULTS
Thyroid autoantibodies (TAAs) were positive in AT/AU (40%), mild AA (14%), and healthy subjects (4%). The frequency of TAAs was significantly higher in patients with AT/AU than in mild AA (p=0.001) and healthy controls (p less than 0.001). The frequency of thyroid peroxidase antibody (TPO-Abs) was significantly higher in patients with AT/AU than in mild AA and healthy controls (p less than 0.001 for both). The frequency of TG-Abs was significantly higher in patients with AT/AU (p=0.003) and mild AA (p=0.043) than in healthy controls. Serum TSH level was significantly higher in AT/AU patients than in mild AA patients (p=0.006) and healthy controls (p=0.005).
CONCLUSION
Severe subtype of AA is associated with a high risk of autoimmune thyroid disease. This highlights the significance of screening for thyroid abnormalities and TAAs in patients with AT/AU.
Topics: Alopecia Areata; Case-Control Studies; Female; Humans; Male; Prospective Studies; Thyroiditis, Autoimmune
PubMed: 27279512
DOI: 10.15537/Smj.2016.6.13777 -
Clinical, Cosmetic and Investigational... 2020Alopecia areata (AA) is an autoimmune disease characterized by the development of non-scarring alopecia. The prevalence is not well known, and estimates vary...
PURPOSE
Alopecia areata (AA) is an autoimmune disease characterized by the development of non-scarring alopecia. The prevalence is not well known, and estimates vary considerably with no recent estimates in the United States (US). The objective of this study was to define the current AA point prevalence estimate among the general population in the US overall and by severity.
PATIENTS AND METHODS
We administered an online, cross-sectional survey to a representative sample of the US population. Participants self-screening as positive for AA using the Alopecia Assessment Tool (ALTO) also completed the Severity of Alopecia Tool (SALT) to measure the severity of disease as a percent of scalp hair loss. Self-reported AA participants were invited to upload photographs for adjudication of AA by 3 clinicians.
RESULTS
The average age of participants was 43 years. Approximately half of the participants (49.2%) were male, and the majority were white (77.1%) and not of Hispanic origin (93.2%). Among the 511 self-reported AA participants, 104 (20.4%) uploaded photographs for clinician evaluation. Clinician-adjudicated point prevalence of AA was 0.21% (95% CI: 0.17%, 0.25%) overall, 0.12% (95% CI: 0.09%, 0.15%) for "mild" disease (≤50% SALT score), and 0.09% (95% CI: 0.06%, 0.11%) for "moderate to severe" disease (>50% SALT score) with 0.04% (95% CI: 0.02%, 0.06%) for the alopecia totalis/alopecia universalis (100% SALT score) "moderate to severe" subgroup. The average SALT score was 44.4% overall, 8.8% for "mild", and 93.4% for "moderate to severe".
CONCLUSION
This study suggests that the current AA prevalence in the US is similar to the upper estimates from the 1970s at approximately 0.21% (700,000 persons) with the current prevalence of "moderate to severe" disease at approximately 0.09% (300,000 persons). Given this prevalence and the substantial impact of AA on quality of life, the burden of AA within the US is considerable.
PubMed: 32280257
DOI: 10.2147/CCID.S245649