New-Onset Rheumatologic Disease in an Elderly Patient Initially Presenting as Worsening Sequelae of Longstanding Peripheral Vascular Disease

Systemic lupus erythematosus (SLE) is an autoimmune disease that is believed to activate and attack nuclear antigens in genetically susceptible individuals after exposure to environmental factors causing cell damage.1,2 Although it is most common in females of child-bearing age, initial presentation is not strictly limited to this population, as onset over the age of 50 years is reported in 3-18% of cases.2 The common manifestations of SLE affect nearly every system of the body and may include arthralgia, myalgia, fever, rash, hepatosplenomegaly, lymphadenopathy, pleuritis, glomerulonephritis, pericarditis and neuropsychiatric manifestations.1-3 Common laboratory findings in SLE with varying degrees of sensitivity and specificity include anti-nuclear antibodies (ANA), anti-doublestranded DNA antibodies (anti-dsDNA), anti-histone antibodies, elevated inflammatory markers, and decreased levels of complements C3 and C4.1 Treatment is typically aimed toward symptom management and prevention of organ damage; thus, treatment regimens are typically dictated by the organ systems involved and symptoms experienced.1 Hydralazine, a vasodilating drug used for treatment of hypertension, has been demonstrated to cause various rheumatologic complications, including a lupus-like syndrome and an anti-neutrophil cytoplastic antibody (ANCA) vasculitis, which tend to present with overlapping features.3-5 Notably, both of these hydralazine-induced rheumatologic diseases tend to present similarly to their primary counterparts but with less severity and less organ involvement; however, hydralazine-induced lupus is particularly prevalent in the elderly population whereas the limited data available regarding hydralazine-induced ANCA vasculitis is inconclusive regarding a predominant age-group affected.3-5 The definitive treatment of both of these complications of hydralazine therapy is early identification and intervention with cessation of hydralazine therapy, given that in both diseases, symptoms typically resolve upon cessation of the offending agent.3,4 With this treatment in mind, cessation of the offending agent can also be diagnostic in terms of determining whether a patient’s clinical presentation is due to primary or drug-induced rheumatologic disease. Here, we present a 78-year-old woman, Mrs. J, who was on long-term hydralazine therapy with apparently worsening complications of her known peripheral artery disease, chronic kidney disease, and type 2 diabetes mellitus and was subsequently found to have several findings concerning for new-onset underlying rheumatologic disease, possibly hydralazine-induced

Carbamazepine Induced Bullous Pemphigoid in a 49 Year Old Male

Bullous pemphigoid is an autoimmune blistering condition mediated by autoantibodies1. It is categorized as an uncommon disorder2-4, with an estimated incidence of 2.4-21.7 cases per million2-6 but carries significant morbidity and mortality, warranting clinical awareness and investigation7,8. A number of medications have been implicated in the development of bullous pemphigoid including loop diuretics, ace inhibitors, and anti-epileptic drugs. This is a case report of carbamazepine-induced bullous pemphigoid in a 49-year-old male after taking the medication for almost 30 years. Diagnosis of bullous pemphigoid was based on biopsy histology and immunofluorescence, as well as the presence of BP 180 antibody. Clinical features of extensive rash and bullae were present on dermatological exam. Upon discontinuation of carbamazepine and appropriate treatment of bullous pemphigoid, the patient’s condition improved. A thorough analysis of the patient’s history and medications did not reveal any other potential triggers of bullous pemphigoid. The only two previous reports of an association between carbamazepine and bullous pemphigoid are limited by lack of immunologic evidence of diagnosis or the identification of a specific causative agent. To address these limitations, we describe what is to our knowledge, the first reported case of clearly documented association between carbamazepine and bullous pemphigoid

From the Editors

It is our honor to present the product of 22 years of resident-run tradition – the 2020-2021 annual edition of The Medicine Forum. In the world of Jefferson traditions, ours is a small one. There is no regalia, pomp and circumstance, or any such fanfare in this marking of the year’s close. Rather than the celebratory release of those other springtime occasions, this publication is a representation of the yearlong dedication and hard work of our residents and fellows in their academic pursuits. We at The Medicine Forum know that producing scholarly work even during what would constitute a normal year can be that added stressor that just feels like too much. In a year where we have continued to see high caseloads of COVID-19 (bearing the emotional toll that comes with it to providers), scrambled to vaccinate as many of our clinic patients as possible against the disease, and tried to balance a world attempting to go back to normal during clearly abnormal times, it amazes us what you all were able to produce. To our submission writers, thank you for sharing in – despite all this – perhaps medicine’s most important practice, the furthering and dissemination of medical knowledge. To our supporters, thank you for making this journal possible. And finally, to our readers, thank you for partaking in this, our small tradition, the 22nd edition of The Medicine Forum

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)

In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to examine autophagy and related processes, and for reviewers to provide realistic and reasonable critiques of reports that are focused on these processes. These guidelines are not meant to be a dogmatic set of rules, because the appropriateness of any assay largely depends on the question being asked and the system being used. Moreover, no individual assay is perfect for every situation, calling for the use of multiple techniques to properly monitor autophagy in each experimental setting. Finally, several core components of the autophagy machinery have been implicated in distinct autophagic processes (canonical and noncanonical autophagy), implying that genetic approaches to block autophagy should rely on targeting two or more autophagy-related genes that ideally participate in distinct steps of the pathway. Along similar lines, because multiple proteins involved in autophagy also regulate other cellular pathways including apoptosis, not all of them can be used as a specific marker for bona fide autophagic responses. Here, we critically discuss current methods of assessing autophagy and the information they can, or cannot, provide. Our ultimate goal is to encourage intellectual and technical innovation in the field

Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356

non present