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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

014 Differential gene expression in lesional skin may signify immune-mediated lung parenchymal damage in dermatomyositis patients [Meeting Abstract]

Shaw, K; Doudican, N; Frazzette, N; Caplan, A; Femia, A; Carucci, J
Dermatomyositis (DM) is an autoimmune connective tissue disease that most commonly affects skin and muscles. Clinical manifestations can be protean, with some patients experiencing skin-limited disease while others develop serious systemic complications including interstitial lung disease (ILD). While both T cell-mediated and humoral immune dysregulation have been purported to play a role in manifesting said end-organ damage, our understanding of immunophenotypic prognostic factors associated with the development of ILD in DM patients remains limited. Thus, we sought to identify potential biomarkers that might be differentially expressed in lesional skin obtained from DM patients with versus without ILD. Utilizing NanoString technology, we assessed differential mRNA expression of 800 immune-related genes in formalin-fixed, paraffin-embedded lesional skin samples obtained from five DM patients with ILD compared to five DM patients without ILD. Among 42 highly regulated genes, 10 were significantly (p<0.05) upregulated in DM patients with ILD: CD44, NFKBIZ, CD24, EGR1, DEFB103B, CCL18, CCL22, CXCL2, S100A8 and S100A9. Notably, serum levels of S100A8/A9 heterodimer (an endogenous ligand of Toll-like receptor 4) are known to correlate with clinical severity in patients with DM-associated ILD, thereby supporting NanoString's utility in identifying salient genes associated with end-organ damage in DM. We also identified several novel genes downregulated in patients with DM-associated ILD that play a role in autophagy and adaptive immune activation: CLEC7, LEF1, KLRC2, BTLA, MUC1, and TCF7. Taken together, our results begin to characterize an immune-related gene expression signature in lesional DM skin that may be associated with comorbid ILD. While validation experiments are ongoing, the identification of biomarkers of systemic disease in lesional DM skin not only has the potential for risk stratifying DM patients at the time of tissue diagnosis but may provide novel targets for early intervention against DM-associated ILD.
ISSN: 1523-1747
CID: 5291982

Prominent dyspigmentation in a patient with dermatomyositis and TIF1-γ autoantibodies [Case Report]

Gutierrez, Daniel; Svigos, Katerina; Femia, Alisa; Brinster, Nooshin K; Lo Sicco, Kristen
PMID: 35355654
ISSN: 2352-5126
CID: 5219942

Clinical Characteristics, Disease Course, and Outcomes of Patients With Acute Generalized Exanthematous Pustulosis in the US

Creadore, Andrew; Desai, Sheena; Alloo, Allireza; Dewan, Anna K; Bakhtiar, Mina; Cruz-Diaz, Carla; Femia, Alisa; Fox, Lindy; Katz, Kimberly L; Micheletti, Robert; Nelson, Caroline A; Ortega-Loayza, Alex G; Patrinely, J Randall; Plovanich, Molly; Rosenbach, Misha; Shaigany, Sheila; Shields, Bridget E; Saleh, Jamal Z; Sharif-Sidi, Zakariyah; Shinkai, Kanade; Smith, Jacob; Su, Chang; Wanat, Karolyn A; Wieser, Jill K; Wright, Shari; Noe, Megan H; Mostaghimi, Arash
Importance/UNASSIGNED:Acute generalized exanthematous pustulosis (AGEP) is a rare, severe cutaneous adverse reaction associated with systemic complications. Currently available data are largely limited to small retrospective case series. Objective/UNASSIGNED:To describe the clinical characteristics, disease course, and outcomes of a heterogeneous group of patients with AGEP across the US. Design, Setting, and Participants/UNASSIGNED:A retrospective review of a case series of patients was conducted from January 1, 2000, through July 31, 2020. All 340 included cases throughout 10 academic health systems in the US were scored retrospectively using the EuroSCAR scoring system, and patients with a score corresponding to probable or definite AGEP and aged 18 years or older were included. Main Outcomes and Measures/UNASSIGNED:Patient demographic characteristics, clinical course, suspected causative agent, treatment, and short- and long-term outcomes. Results/UNASSIGNED:Most of the 340 included patients were women (214 [62.9%]), White (206 [60.6%]), and non-Hispanic (239 [70.3%]); mean (SD) age was 57.8 (17.4) years. A total of 154 of 310 patients (49.7%) had a temperature greater than or equal to 38.0 °C that lasted for a median of 2 (IQR, 1-4) days. Of 309 patients, 263 (85.1%) developed absolute neutrophilia and 161 patients (52.1%) developed either absolute or relative eosinophilia. Suspected causes of AGEP were medications (291 [85.6%]), intravenous contrast agents (7 [2.1%]), infection (3 [0.9%]), or unknown (39 [11.5%]). In 151 cases in which a single medication was identified, 63 (41.7%) were β-lactam antimicrobials, 51 (33.8%) were non-β-lactam antimicrobials, 9 (6.0%) were anticonvulsants, and 5 (3.3%) were calcium channel blockers. The median time from medication initiation to AGEP start date was 3 (IQR, 1-9) days. Twenty-five of 298 patients (8.4%) had an acute elevation of aspartate aminotransferase and alanine aminotransferase levels, with a peak at 6 (IQR, 3-9) days. Twenty-five of 319 patients (7.8%) experienced acute kidney insufficiency, with the median time to peak creatinine level being 4 (IQR, 2-5) days after the AGEP start date. Treatments included topical corticosteroids (277 [81.5%], either alone or in combination), systemic corticosteroids (109 [32.1%]), cyclosporine (10 [2.9%]), or supportive care only (36 [10.6%]). All-cause mortality within 30 days was 3.5% (n = 12), none of which was suspected to be due to AGEP. Conclusions and Relevance/UNASSIGNED:This retrospective case series evaluation of 340 patients, the largest known study cohort to date, suggests that AGEP onset is acute, is usually triggered by recent exposure to an antimicrobial, may be associated with liver or kidney complications in a minority of patients, and that discontinuation of the triggering treatment may lead to improvement or resolution.
PMID: 34985493
ISSN: 2168-6084
CID: 5107142

Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States

Noe, Megan H; Wan, Marilyn T; Mostaghimi, Arash; Gelfand, Joel M; Agnihothri, Ritesh; Armstrong, April W; Bhutani, Tina; Bridges, Alina; Brownstone, Nicholas; Butt, Melissa; Duffin, Kristina P Callis; Carr, Christian; Creadore, Andrew; DeNiro, Katherine L; Desai, Sheena; Dominguez, Arturo R; Duffy, Emily K; Fairley, Janet A; Femia, Alisa; Gudjonsson, Johann E; Kaffenberger, Jessica A; Katz, Kimberly L; Le, Stephanie T; Martinez, Edgar; Maverakis, Emanual; Myers, Bridget; Naik, Haley B; Nelson, Caroline A; Ortega-Loayza, Alex G; Plovanich, Molly E; Rangel, Lauren K; Ravi, Vignesh; Reddy, Vidhatha D; Saleh, Jamal Z; Kirby, Joslyn S; Sandhu, Jeena K; Shakshouk, Hadir; Shields, Bridget E; Sharif-Sidi, Zakariyah; Smith, Jacob; Steahr, Amanda; Toussi, Atrin; Wanat, Karolyn A; Wang, Bo; Wei, Brian M; Weinhammer, Annika; Worswick, Scott D; Yang, Alexander
Importance/UNASSIGNED:Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. Objective/UNASSIGNED:To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. Design, Setting, and Participants/UNASSIGNED:A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. Main Outcomes and Measures/UNASSIGNED:The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. Results/UNASSIGNED:Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). Conclusions and Relevance/UNASSIGNED:Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.
PMID: 34878491
ISSN: 2168-6084
CID: 5110252