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• W2283513761 abstract "To the Editor: Linear morphea (LM) is characterized by linear sclerotic plaques on the limbs or face and scalp. The disorder predominantly manifests in the pediatric population and often results in functional disability or permanent disfigurement. In 2012, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) recommended methotrexate (MTX) with or without systemic corticosteroids (CS) as first-line therapy for pediatric-onset LM.1Li S.C. Torok K.S. Pope E. et al.Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma.Arthritis Care Res (Hoboken). 2012; 64: 1175-1185PubMed Google Scholar However, little is known about adult-onset LM. Thus, we sought to describe the characteristics and treatment of adult-onset LM at 3 tertiary care centers. After IRB approval, we used a natural language search and ICD-9 code 701.0 (“circumscribed scleroderma”) to query the Partners Research Patient Data Registry (1995-2015; Brigham and Women's and Massachusetts General Hospitals) and two medical record systems at New York University (2005-2014), together including more than 20 million patient visits. All patients with LM onset at 18 years or older were included. Data were extracted on demographics, disease presentation, and treatment regimens (as defined in Table I). Clinical response (as defined in Table II) was determined by medical record review and available clinical photographs.Table IDemographics, clinical features, and treatments of patients with adult-onset linear morpheaCharacteristicN (%)(unless noted otherwise)Age at onset in years, mean (SD) (N = 59)∗Two patients developed LM after 20 years of age, but lacked documentation of an exact age and thus were excluded from the mean age of onset calculation.35 (13)Sex (N = 61) Female51 (84) Male10 (16)Race (N = 51) White37 (72) Hispanic10 (20) Asian2 (4) Black2 (4)Type of LM (N = 61) ECDS33 (54) Extremity LM20 (33) HFA7 (11) ECDS and extremity LM1 (2)Interval between symptom onset and diagnosis of LM by a dermatologist in months, (SD) (N = 45)27 (47)Joint contractures in extremity LM (N = 18)8 (44)Physical therapy for joint contractures in extremity LM (N = 8)4 (50)Brain MRI performed in ECDS or HFA (N = 41)17 (41)MRI evidence of brain involvement in ECDS or HFA (N = 17)3 (18)Evaluation to exclude ocular involvement in ECDS or HFA (N = 40)9 (23)Documentation of self-reported psychological distress in ECDS or HFA (N = 41)9 (22)Coexisting autoimmune conditions (N = 61) Lupus erythematosus4 (7) Hashimoto's thyroiditis3 (5) Sjögren's syndrome2 (3) Rheumatoid arthritis1 (2) Relapsing polychondritis1 (2) Antiphospholipid syndrome1 (2)Treatment regimens (N = 113)†A treatment regimen was defined as monotherapy, combination therapy, or no therapy pursued until the development of a clinical response. using MTX with systemic CS10 (9) MTX without systemic CS16 (14) Systemic CS without MTX6 (5) MMF2 (2) Phototherapy8 (7)UVA12 (2)PUVA2 (2)NBUVB4 (4) Antimalarials15 (13) Other agents‡Tetracyclines (N = 25), topical CS (N = 22), topical vitamin D analogs (N = 18), topical calcineurin inhibitors (N = 18), intralesional CS (N = 6), calcitriol (N = 9), unspecified topical agents (N = 3), azelaic acid (N = 3), dipyridamole (N = 2), infliximab (N = 1), colchicine (n = 1), potassium aminobenzoate (N = 1), topical dapsone (N = 1), topical tretinoin (N = 2), ofloxacin (N = 1), hydroquinone (N = 1), topical vitamin E (N = 1), petroleum jelly (N = 1), and saline soaks (N = 1).67 (59) No treatment5 (4)Follow-up in months, mean (SD) (N = 57)38 (50)N for each characteristic reflects the total number of patients with data on the characteristic or, in the case of treatment regimens, the total number of regimens.CS, Corticosteroids; ECDS, en coup de sabre; HFA, hemifacial atrophy; LM, linear morphea; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; MTX, methotrexate; NBUVB, narrowband ultraviolet B therapy; PUVA, psoralen ultraviolet A therapy; UVA1, ultraviolet A1 therapy.∗ Two patients developed LM after 20 years of age, but lacked documentation of an exact age and thus were excluded from the mean age of onset calculation.† A treatment regimen was defined as monotherapy, combination therapy, or no therapy pursued until the development of a clinical response.‡ Tetracyclines (N = 25), topical CS (N = 22), topical vitamin D analogs (N = 18), topical calcineurin inhibitors (N = 18), intralesional CS (N = 6), calcitriol (N = 9), unspecified topical agents (N = 3), azelaic acid (N = 3), dipyridamole (N = 2), infliximab (N = 1), colchicine (n = 1), potassium aminobenzoate (N = 1), topical dapsone (N = 1), topical tretinoin (N = 2), ofloxacin (N = 1), hydroquinone (N = 1), topical vitamin E (N = 1), petroleum jelly (N = 1), and saline soaks (N = 1). Open table in a new tab Table IIClinical response of adult-onset linear morphea to treatment regimens with or without methotrexateClinical responseRegimens with MTX∗Excludes regimens without known clinical response.(N = 24)N (%)Regimens without MTX∗Excludes regimens without known clinical response.(N = 79)N (%)P valueCategorical variables were compared using two-tailed χ2 tests; P ≤ .05 was considered statistically significant.Progression†Defined as new lesion development or existing lesion extension.1 (4)27 (34).004Stabilization‡Defined as lack of lesion development and extension without lesion improvement.10 (42)23 (29).248Improvement§Defined as partial or complete improvement of erythema, joint contractures, induration, and/or atrophy.13 (54)29 (37).127Resolution¶Defined as complete improvement of erythema, joint contractures, induration, and/or atrophy.7 (29)3 (4)<.001Flare‖Defined as disease reactivation after pharmacologic stabilization or improvement, or after quiescence without treatment.#Denominator used to calculate percentages excludes regimens resulting in disease progression.3 (13)29 (56)<.001No flare#Denominator used to calculate percentages excludes regimens resulting in disease progression.20 (87)22 (42)<.001Flare unknown#Denominator used to calculate percentages excludes regimens resulting in disease progression.0 (0)1 (2).503MTX, Methotrexate.∗ Excludes regimens without known clinical response.† Defined as new lesion development or existing lesion extension.‡ Defined as lack of lesion development and extension without lesion improvement.§ Defined as partial or complete improvement of erythema, joint contractures, induration, and/or atrophy.¶ Defined as complete improvement of erythema, joint contractures, induration, and/or atrophy.‖ Defined as disease reactivation after pharmacologic stabilization or improvement, or after quiescence without treatment.# Denominator used to calculate percentages excludes regimens resulting in disease progression.∗∗ Categorical variables were compared using two-tailed χ2 tests; P ≤ .05 was considered statistically significant. Open table in a new tab N for each characteristic reflects the total number of patients with data on the characteristic or, in the case of treatment regimens, the total number of regimens. CS, Corticosteroids; ECDS, en coup de sabre; HFA, hemifacial atrophy; LM, linear morphea; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; MTX, methotrexate; NBUVB, narrowband ultraviolet B therapy; PUVA, psoralen ultraviolet A therapy; UVA1, ultraviolet A1 therapy. MTX, Methotrexate. Sixty-one patients with adult-onset LM were identified (Table I). The mean delay in diagnosis, defined as the interval between symptom onset and diagnosis of LM by a dermatologist, was 27 months. Forty-four percent of patients with extremity LM had functional limitations; only half were referred for physical therapy. One patient had limb length discrepancy. Among en coup de sabre or hemifacial atrophy patients, 41% had magnetic resonance imaging of the brain, and 23% underwent ophthalmologic evaluation. MTX with or without systemic CS was used in 23% of treatment regimens. Disease resolution was significantly more likely (29% vs 4%, P < .001), and progression (4% vs 34%, P = .004) and recurrence (13% vs 56%, P < .001) significantly less likely, with treatment regimens including MTX as compared to those without, during a mean follow-up of 38 months (Table II). This study represents the largest adult-onset LM cohort to date, and is the first to analyze both the characteristics and management of adult-onset LM.2Mertens J.S. Seyger M.M. Kievit W. et al.Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease.Br J Dermatol. 2015; 172: 722-728Crossref PubMed Scopus (45) Google Scholar, 3Arkachaisri T. Fertig N. Pino S. Medsger Jr., T.A. Serum autoantibodies and their clinical associations in patients with childhood- and adult-onset linear scleroderma. A single-center study.J Rheumatol. 2008; 35: 2439-2444Crossref PubMed Scopus (68) Google Scholar, 4Condie D. Grabell D. Jacobe H. Comparison of outcomes in adults with pediatric-onset morphea and those with adult-onset morphea: a cross-sectional study from the morphea in adults and children cohort.Arthritis Rheumatol. 2014; 66: 3496-3504Crossref Scopus (47) Google Scholar Delayed diagnosis and permanent functional sequelae were common. Referrals for physical therapy, neurologic imaging, and evaluation of ocular involvement were underused. Treatment regimens incorporating MTX were more likely to result in resolution and less likely to result in progression or reactivation. This is notable given that flares in LM are common regardless of age at disease onset.2Mertens J.S. Seyger M.M. Kievit W. et al.Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease.Br J Dermatol. 2015; 172: 722-728Crossref PubMed Scopus (45) Google Scholar While MTX is considered first-line for pediatric-onset LM, this study is the first to support its use for adult-onset LM. Nevertheless, in this study, less than a quarter of treatment regimens included MTX. Although our study period largely predates the CARRA guidelines, a 2015 national survey found that physicians treating adults with LM were more likely to prescribe topical CS or phototherapy, whereas those treating children were more likely to prescribe MTX.5Strickland N. Patel G. Strickland A. Jacobe H. Attitudes and trends in the treatment of morphea: a national survey.J Am Acad Dermatol. 2015; 72: 727-728Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar Thus, MTX appears to be underused in the treatment of adult-onset LM. This study's limitations include its retrospective nature and lack of objective outcome measures in patients treated at various disease stages. Despite the small sample size, this study represents the largest adult-onset LM cohort to date. Further investigation is needed to determine a treatment algorithm for adult-onset LM." @default.
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• W2283513761 title "Characteristics and treatment of adult-onset linear morphea: A retrospective cohort study of 61 patients at 3 tertiary care centers" @default.
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