ABSTRACT
This cohort study examines the association between methotrexate use and interstitial lung disease in patients with dermatomyositis.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Methotrexate , Humans , Dermatomyositis/drug therapy , Dermatomyositis/chemically induced , Dermatomyositis/diagnosis , Lung Diseases, Interstitial/chemically induced , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/drug therapy , Methotrexate/adverse effects , Methotrexate/administration & dosage , Female , Middle Aged , Male , Adult , Dermatologic Agents/adverse effects , Dermatologic Agents/administration & dosage , Risk Factors , AgedABSTRACT
This cohort study describes the clinical features, patient characteristics, and treatment of anti-melanoma differentiationassociated gene 5 (MDA5) dermatomyositis.
Subject(s)
Continuity of Patient Care , Dermatomyositis , Hospitalization , Interferon-Induced Helicase, IFIH1 , Humans , Dermatomyositis/immunology , Dermatomyositis/drug therapy , Dermatomyositis/therapy , Interferon-Induced Helicase, IFIH1/immunology , Hospitalization/statistics & numerical data , Female , Continuity of Patient Care/organization & administration , Male , Middle Aged , Adult , Autoantibodies/blood , Autoantibodies/immunologySubject(s)
Dermatitis , Population Health , Scleroderma, Localized , Adult , Humans , Scleroderma, Localized/epidemiology , Skin , ComorbidityABSTRACT
This cohort study characterizes the presentation, causes, treatment, and disease course of erythema nodosum, as well as identifies associations with chronicity and recurrence.
Subject(s)
Erythema Nodosum , Humans , Erythema Nodosum/diagnosis , Erythema Nodosum/complications , RecurrenceSubject(s)
Atrial Fibrillation , Cardiovascular Diseases , Dermatomyositis , Myocardial Infarction , Population Health , Stroke , Humans , Cross-Sectional Studies , Dermatomyositis/epidemiology , Dermatomyositis/complications , Comorbidity , Myocardial Infarction/epidemiology , Atrial Fibrillation/complications , Risk Factors , Stroke/epidemiology , Cardiovascular Diseases/epidemiology , Cardiovascular Diseases/complicationsABSTRACT
ABSTRACT: The Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway plays a critical role in the pathogenesis of many immune-mediated inflammatory diseases (IMIDs). Although Janus kinase inhibitors (JAKi) are an effective treatment for several IMIDs, they have come under scrutiny as a class due to a potential risk of venous thromboembolism (VTE) and cardiovascular (CV) events, specifically noted with the oral JAKi, tofacitinib, as reported in the ORAL Surveillance Trial of a high CV risk rheumatoid arthritis population. This trial resulted in a black box warning from the Food and Drug Administration and European Medicines Agency regarding risk of VTE and CV events that was extended across several types of JAKi (including topical ruxolitinib) when treating IMIDs, leading to considerable controversy. Included is an up-to-date review of the current and rapidly evolving literature on CV risk in patients with IMIDs on JAKi therapy, including identification of potential risk factors for future VTE and CV events on JAKi therapy. We suggest a comprehensive, multimodal, and systematic approach for evaluation of CV risk in patients considering taking JAKi and emphasize that cardiologists play an important role in risk stratification and mitigation for patients with high CV risk factors or on long-term JAKi therapies.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Skin Diseases , Humans , Dermatomyositis/genetics , Lung , Gene ExpressionABSTRACT
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.
Subject(s)
Pyoderma Gangrenosum , Humans , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/epidemiology , Pyoderma Gangrenosum/therapy , Retrospective Studies , Immunosuppressive Agents/therapeutic use , Skin/pathology , Adrenal Cortex HormonesABSTRACT
Evidence-based literature regarding management of rare and severe dermatologic disease is limited. Canakinumab and anakinra, two therapeutics used for inhibiting IL-1 pathways, have seen increased utilization for treatment of refractory dermatoses. We sought to better characterize the breadth of dermatologic conditions for which these medications could be utilized.
Subject(s)
Antibodies, Monoclonal, Humanized/therapeutic use , Interleukin 1 Receptor Antagonist Protein , Interleukin-1 , Skin Diseases/drug therapy , Humans , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Interleukin-1/antagonists & inhibitors , Treatment OutcomeABSTRACT
Importance: 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: To describe the clinical characteristics, disease course, and outcomes of a heterogeneous group of patients with AGEP across the US. Design, Setting, and Participants: 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: Patient demographic characteristics, clinical course, suspected causative agent, treatment, and short- and long-term outcomes. Results: 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 ß-lactam antimicrobials, 51 (33.8%) were non-ß-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: 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.
Subject(s)
Acute Generalized Exanthematous Pustulosis , Acute Generalized Exanthematous Pustulosis/diagnosis , Acute Generalized Exanthematous Pustulosis/etiology , Adolescent , Anti-Bacterial Agents/adverse effects , Female , Glucocorticoids , Humans , Middle Aged , Retrospective Studies , SkinABSTRACT
IMPORTANCE: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: 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: 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: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. RESULTS: 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: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.