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Autoimmune and Cutaneous Inflammatory Comorbidities in Adult-Onset Morphea in the All of Us Research Program [Letter]

Shah, Jill T; Richardson, William Mark; Mittal, Lavanya; Hejazi, Emily; Mazori, Daniel R; Femia, Alisa N
PMID: 38305944
ISSN: 1179-1888
CID: 5626922

Erythema Ab Igne: Decoding Skin Presentations of Vasculitis Mimickers in Autoimmunity

Buontempo, Michael G; Ramachandran, Vignesh; Mazori, Daniel R; Femia, Alisa; Sikora, Michelle; Sicco, Kristen Lo; Caplan, Avrom S
PMID: 37871732
ISSN: 1555-7162
CID: 5614292

Clinical Characteristics of Erythema Nodosum and Associations With Chronicity and Recurrence

Shah, Jill T; Richardson, William Mark; Caplan, Avrom S; Mazori, Daniel R; Femia, Alisa N
PMID: 38170490
ISSN: 2168-6084
CID: 5628332

Utility of magnetic resonance imaging in the diagnosis of eosinophilic fasciitis: A multicenter retrospective cohort study

Shahriari, Neda; Mazori, Daniel R; Shahriari, Mona; Taylor, Dustin; Shaw, Katharina; LaChance, Avery H; Femia, Alisa N; Vleugels, Ruth Ann
PMID: 37659455
ISSN: 1097-6787
CID: 5609322

Intravenous Immunoglobulin for Refractory Eosinophilic Fasciitis: A Retrospective Analysis from 3 Tertiary Care Centers

Tkachenko, Elizabeth; Steuer, Alexa B; Lo, Kelly; Cobos, Gabriela; Lo Sicco, Kristen; Vleugels, Ruth Ann; Femia, Alisa N
PMID: 31846716
ISSN: 1097-6787
CID: 4242452

Differential gene expression in lesional skin may signify immune-mediated lung parenchymal damage in patients with dermatomyositis

Shaw, Katharina; Doudican, Nicole; Mishra, Arnav; Frazzette, Nicholas; Caplan, Avrom S; Femia, Alisa; Carucci, John
PMID: 36641011
ISSN: 1097-6787
CID: 5426332

Dermatomyositis Diagnosis and Treatment in the Inpatient Setting

Hejazi, Emily Z.; Mittal, Lavanya; Sicco, Kristen Lo; Mazori, Daniel R.; Femia, Alisa N.; Caplan, Avrom S.
Purpose of Review: Dermatomyositis can present with a range of manifestations and severity that may necessitate hospital admission. Dermatologists are frequently consulted for patients with dermatomyositis inpatient. Herein we describe clinical features and management of multisystem complications of dermatomyositis with a focus on the inpatient setting. Recent Findings: Patients with dermatomyositis are at risk for hospitalization due to disease flares, infections, and systemic complications. Furthermore, patients may seek care for symptoms including shortness of breath, fever, or cutaneous eruptions which can lead to a new diagnosis of dermatomyositis. Patients with dermatomyositis have increased healthcare utilization and necessitate multidisciplinary and collaborative care. Cutaneous findings may be subtle yet provide important prognostic information. Symptoms arising from skin disease may also be chronic and refractory. Summary: Dermatologists are essential in both diagnosing and managing dermatomyositis and must be attuned to the multiple systemic manifestations and complications that impact inpatient care.
ISSN: 2162-4933
CID: 5499992

Comment on "Skin disease of the breast and nipple" [Letter]

Gutierrez, Daniel; Steuer, Alexa B; Pomeranz, Miriam K; Femia, Alisa N
PMID: 31972256
ISSN: 1097-6787
CID: 4273982

Diagnostic work-up and treatment in patients with pyoderma gangrenosum: retrospective analysis of US insurance claims-based data

Shaigany, Sheila; Wong, Priscilla W; Caplan, Avrom; Kim, Randie H; Femia, Alisa
Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and "real-world" practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.
PMID: 34714405
ISSN: 1432-069x
CID: 5042872

Association of Dermatomyositis with Cardiovascular Disease: A Case-Control Study in the All of Us Research Program [Meeting Abstract]

Shah, J; Shah, K; Mazori, D; Caplan, A; Hejazi, E; Femia, A
Background/Purpose: Previous studies on the association of dermatomyositis (DM) with cardiovascular (CV) disease have used combined idiopathic inflammatory myositis cohorts, included only non-United States (US) cohorts, included only inpatients, or have not included matched controls. We aimed to describe the burden and timing of CV disease in a demographically and geographically diverse sample of inpatients and outpatients with DM in the US. Table 1. All of Us Database Diagnosis Search Terms.
Method(s): We performed a nested, matched, case-control analysis based on diagnostic coding in the All of Us Registered Tier Dataset v5 (Table 1). We used nearest neighbor propensity score matching to select for age-, sex-, race-, and ethnicity-matched controls for each DM case. We compared CV comorbidities and their dates of diagnosis between cases and controls using Pearson's chi-squared test or Fisher's exact test for categorical variables and the unpaired t-test for continuous variables. A multivariable conditional logistic regression model was built by including comorbidities with significance of P < 0.1 in univariable analysis, followed by backward elimination of comorbidities with a significance of P > 0.1 or with evidence of collinearity. A sensitivity analysis was performed that excluded DM cases with comorbid systemic lupus Table 2. Demographic and clinical characteristics of DM cases versus age-, sex-, race-, and ethnicity-matched controls in All of Us. erythematosus (SLE), rheumatoid arthritis, psoriasis, or systemic sclerosis.
Result(s): Of the 214,206 All of Us participants with electronic health record data, we identified 248 DM cases and 992 controls (Table 2). The mean follow-up time for DM cases was 7.1 +/- 4.8 years. Compared to controls, DM cases were significantly associated with 14 of 14 tested CV comorbidities in univariable analysis: atrial fibrillation (AF), cerebrovascular disease (CVD), chronic kidney disease (CKD), chronic obstructive pulmonary disease, coronary artery disease, deep vein thrombosis, heart failure, hyperlipidemia, hypertensive disorder (HTN), myocardial infarction, peripheral artery disease, pulmonary embolism, type 2 diabetes (T2D), and valvular heart disease (VHD). Aside from HTN, which was diagnosed on average 3.6 years earlier in the DM cohort, comorbidities were diagnosed at similar ages between cases and controls. In multivariable analysis, CKD, CVD, T2D, and VHD remained significantly associated with DM (Table 3). In the sensitivity analysis, 154 cases and 616 controls were identified. Univariable analysis results were similar except AF was not a significant association. In multivariable analysis, CKD, T2D, and VHD remained significantly associated; the odds ratio for CVD was 2.11 (p = 0.086).
Conclusion(s): This study found an association between DM and T2D, which has been previously reported. Unique to this study is the strong association of DM with CKD and with VHD, which remained significant in multiple multivariable models. Elevated risk of CV disease has been established in chronic inflammatory states such as SLE. This study shows a similar association between CV disease and DM. It is necessary to establish if treatment of DM decreases risk of CV disease, as is the case in the treatment of other rheumatologic diseases. Our study is limited by ascertainment of diagnoses using electronic health records and a lack of data on clinical features of DM
ISSN: 2326-5205
CID: 5513132