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Sickle cell trait (SCT) has long been considered a benign carrier state with malarial protection, but carriers can be affected by increased venous thromboembolism, exercise-related injury, renal complications and very rarely a fatal renal malignancy. Renal medullary carcinoma is a very rare and aggressive renal tumor described almost exclusively in sickle cell trait. A review of the current literature provides clues to this link and describes trends expected in these cases. We report a case of renal medullary carcinoma in a 32-year-old female with known sickle trait who presented with cough, hemoptysis, left flank pain and gross hematuria. Initial presentation was concerning for pulmonary renal syndrome, but her labs did not show evidence of nephritic syndrome with negative autoimmune and infectious serologies. Abdominal CT imaging identified a large left renal mass with biopsy confirmation of renal medullary carcinoma and subsequent staging showing pulmonary and osseous metastases. Despite palliative chemotherapy, she died within 3 months of diagnosis following a protracted clinical course. Renal medullary carcinoma should be considered in patients with SCT presenting with hematuria.
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This is part of a series of case reports detailing scenarios from our community hospital. The cases are selected to feature clinical dilemmas, provide a review on what is currently known about the topic with expert perspective. A 66-year-old black man presented to the primary care clinic with his fourth episode of generalized painful rash and oral ulcerations without a diagnosis despite two emergency room (ED) visits alongside doxycycline treatment. Symptoms interfered with daily activities with skin exam notable for widespread erythematous patches and plaques. In office biopsies were obtained with final diagnosis of erythema multiforme. Etiologic workup revealed positive HSV 1 IgG and active untreated hepatitis C infection. He was treated with prednisone, and acyclovir with resolution of lesions and no further recurrences over a nine-month follow up period. This case depicts an unusual presentation of a common skin disorder. It highlights the challenge of recognizing atypical target lesions in skin of color and the important role of primary care in bridging access to dermatological care.
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Rheumatoid vasculitis is a rare and late complication of rheumatoid arthritis, affecting small to medium sized vessels. Inflammation in the vessel wall produces an occlusive vasculopathy with downstream infarction. We present the case of a 70-year-old female with long standing stable RA status post recent COVID-19 vaccination admitted with community acquired Legionnaires disease found to have an incidental renal infarct on chest imaging without a clear etiology. She improved from a respiratory standpoint but then had a fever spike with repeat imaging showing a second renal infarct and CT angiography showing extensive beading with aneurysmal dilation in the bilateral hepatic and renal arteries consistent with vasculitis. Given extensive RA history, there was a high index of suspicion for rheumatoid vasculitis and she was treated with pulse steroids followed by Rituximab with tapering of steroids and clinical recovery. Her presentation is unique given the salient features, extensive multi-vessel disease without localizing symptoms and potential role of Legionella infection or COVID-19 vaccine in immune activation.
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Phthalates (PAEs) are a group of synthetic esters of phthalic acid compounds mostly used as plasticizers in plastic materials but are widely applied in most industries and products. As plasticizers in plastic materials, they are not chemically bound to the polymeric matrix and easily leach out. Logically, PAEs should be prevalent in the environment, but their prevalence, transport, fate, and effects have been largely unknown until recently. This has been attributed, inter alia, to a lack of standardized analytical procedures for identifying them in complex matrices. Nevertheless, current advancements in analytical techniques facilitate the understanding of PAEs in the environment. It is now known that they can potentially impact ecological and human health adversely, leading to their categorization as endocrine-disrupting chemicals, carcinogenic, and liver- and kidney-failure-causing agents, which has landed them among contaminants of emerging concern (CECs). Thus, this review article reports and discusses the developments and advancements in PAEs' standard analytical methods, facilitating their emergence from obscurity. It further explores the opportunities, challenges, and limits of their advancements.
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Purpose of review-To review autoantibodies associated with different subtypes of idiopathic inflammatory myopathy (IIM) and their clinical applications. IIM are a heterogenous group of autoimmune disorders characterized by muscle weakness, cutaneous features, and internal organ involvement. The diagnosis and classification, which is often challenging, is made using a combination of clinical features, muscle enzyme levels, imaging, and biopsy. The landmark discoveries of novel autoantibodies specific to IIM subtypes have been one of the greatest advancements in the field of myositis. The specificity of these autoantibodies has simplified the diagnostic algorithm of IIM with their heterogenous presentation and outdated the earlier diagnostic criteria. Myositis-specific antibodies (MSAs) have improved diagnostics, clinical phenotyping, and prognostic stratification of the subtypes of IIMs. Furthermore, the levels of certain MSAs correlate with disease activity and muscle enzyme levels such that titers may be able to be used to predict disease course and treatment response.
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Giant cell arteritis (GCA), or temporal arteritis, is a medium to large vessel vasculitis seen in the elderly. Its presentation varies from fever of unknown origin to cranial ischemic complications including complete vision loss. The early initiation of steroids is key to preventing complications of GCA. Here we discuss the role of aspirin in the treatment of GCA, both as an antithrombotic agent and its increasingly utilized anti-inflammatory properties. The aim of this review article is to examine the evidence behind the use of aspirin as an adjunct to steroids for the prevention of cranial ischemic complications.
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Systemic lupus erythematosus (SLE) is an autoimmune disease with a myriad of clinical presentations and periodic flares. We present a case of a young lady with a history of SLE who presented with constitutional symptoms 1 week after starting Isoniazid and Rifampin for treatment of latent TB. Her presentation shared similarities with several diseases including TB lymphadenitis, SLE flare, Kikuchi-Fujimoto Disease (KFD) and hemophagocytic lymphohistiocytosis (HLH) posing a diagnostic dilemma. Additionally, she presented not long after the onset of the global COVID-19 pandemic, further expanding the differential diagnosis. She was ultimately diagnosed with a severe SLE flare caused by rifampin induced suppression of the CYP3A4 system, thereby reducing the therapeutic efficacy of steroids. This case highlights the deadly potential of drug-drug interactions, especially in patients with autoimmune conditions.
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Lupus enteritis is a rare manifestation seen in systemic lupus erythematosus (SLE). Its diagnosis can be challenging as symptoms frequently overlap many gastrointestinal disorders, imaging findings are not specific, and endoscopic features are infrequently diagnostic. Moreover, enteritis can occur in isolation without other systemic manifestations or even elevated inflammatory markers.1 Here is presented the case of a 22-year-old female with known SLE manifested by lupus nephritis complicated by end-stage renal disease who presented with abdominal pain. She had leukocytosis with thickened bowel loops, ascites, "target sign", "comb sign" and patent abdominal vessels on CT imaging. The differential diagnoses considered ranged from infectious gastroenteritis to systemic vasculitis. Her infectious workup was negative while inflammatory markers and autoimmune workup did not support active lupus flare. Having ruled out alternative etiologies, steroid dosing was increased in consultation with rheumatology. Subsequently, her abdominal pain responded supporting a diagnosis of lupus enteritis. The case was perplexing in light of her non-specific presenting symptoms and the absence of laboratory evidence of active lupus flare which delayed the diagnosis. This case illustrates how the diagnosis of lupus enteritis continues to remain a challenge.