The Great Imitator: A Case of Multisystem Sarcoidosis Involving the Genitourinary System.

Sarcoidosis is a multisystem noncaseating granulomatous disease, which primarily involves the lungs, skin, and lymph nodes. In this case, we describe a 49-year-old Caucasian male presenting with weakness and symptomatic hypercalcemia. Initial workup revealed multiple testicular hypoechoic lesions on ultrasound and pulmonary nodules with hilar lymphadenopathy on a CT scan. Given the age of the patient, the initial differential diagnosis included lymphoma and testicular cancer. However, a lymph node biopsy confirmed the presence of noncaseating granulomas, and thus a diagnosis of multisystem sarcoidosis was made. Treatment with systemic steroids resulted in significant improvement, and he was initiated on methotrexate as a steroid-sparing agent. This case report details an unusual presentation of this multisystemic disease, which infrequently involves the genitourinary system, and presents a review of the literature on the "great imitator."

Thyrotoxic Periodic Paralysis: A Case Report and Literature Review.

Thyrotoxic periodic paralysis (TPP) is a rare presentation of thyrotoxicosis most commonly associated with Graves' disease. It is rare in Caucasians, but it affects approximately 2% of Asians (occurring in those of Chinese, Japanese, Vietnamese, Filipino, and Korean descent) with thyrotoxicosis of any cause. Typical thyrotoxic features may be absent despite biochemical thyrotoxicosis. Hypokalemia and muscle paralysis are the result of an acute intracellular shift of potassium and not due to total body potassium deficiency. TPP is a self-limiting condition that is easily corrected by treatment of the thyrotoxicosis. We present a case of a Filipino man, aged 47 years, who presented to the emergency department with acute bilateral lower extremity weakness and hypokalemia who was subsequently diagnosed with TPP due to Graves' disease.

Synovial fluid cell count of 84 930/mm3 in adult-onset Still's disease.

In traditional teaching, synovial fluid cell counts over 50 000/mm3 are in keeping with septic or crystalline arthritis. We report a patient with adult-onset Still's disease (AOSD) and associated inflammatory arthritis whose synovial fluid cell count was over 80 000/mm3 This is the highest level reported in the literature, demonstrating that in the appropriate clinical context, markedly elevated synovial cell count levels over 50 000/mm3 can be caused by inflammatory conditions like AOSD. Recognition of this is crucial to avoid inappropriate antibiotics and procedures while also ensuring correct immunosuppressive treatments are offered in a timely manner.

Counselling patients for return to work on immunosuppression: practices of Canadian specialists during the COVID-19 pandemic.

OBJECTIVES: The COVID-19 pandemic has infected over 870,000 Canadians and caused 22,000 deaths. Many patients are attempting to balance health and financial stability. Therefore, we sought to determine how physicians who frequently prescribe immunosuppressive medications are counselling patients on return-to-work prior to widespread vaccine distribution and understand their decision processes. METHODS: We administered a survey through the Canadian Rheumatology, Gastroenterology and Dermatology Associations. Physicians were asked whether patients have requested counselling on return-to-work during the pandemic and how they decide what advice to provide. They were shown seven clinical scenarios of patients on immunosuppressive medications, then asked whether they would provide a medical note advocating for delayed return-to-work or modified duties to reduce exposure. RESULTS: 151 physicians took the survey. 94% were asked for advice on return-to-work. 33% felt informed enough to provide counselling. When patients requested a medical note, physicians provided one 25% of the time. Factors most associated with providing notes were patient comorbidities, age, glucocorticoids, high risk work and vulnerable co-inhabitants. Conventional synthetic and biologic immunosuppressants did not prompt most physicians to provide a note. Respondents considered patient perspectives and workplace factors. Several requested guidelines to approach these encounters. CONCLUSIONS: Almost all rheumatologists, dermatologists and gastroenterologists have been asked to counsel patients on returning to work during the COVID-19 pandemic. Most do not feel informed enough to do so. Medical notes for accommodations are only provided a minority of the time, unless specific factors (e.g. glucocorticoids) are present. Guidance is needed to inform these decisions.

Cardiovascular risk management in rheumatoid arthritis: A large gap to close.

OBJECTIVE: Rheumatoid arthritis (RA) portends significant cardiovascular morbidity and mortality. We therefore determined how often rheumatologists screened for and managed cardiovascular risk factors in RA patients, and the barriers to doing so. METHODS: We examined 300 patient charts from 10 university-affiliated rheumatology practices, to ascertain if they had been screened, treated and/or referred over a 3-year period. We subsequently distributed a national survey to Canadian rheumatologists to elucidate challenges in performing optimal cardiovascular risk modification. RESULTS: Most patients were screened for hypertension. Forty-one per cent were found to be hypertensive; however, the majority of these patients were neither treated nor referred to another provider for management. A small minority of patients were screened for diabetes and/or hyperlipidaemia, and these were usually not addressed if abnormal. Men were referred more frequently than women. Consistent with these findings, the majority of rheumatologists from the national survey felt that they did not manage cardiovascular risk adequately; 79.4% cited a lack of time as a major barrier, and 82.5% felt that it should be managed by the primary care provider. CONCLUSION: There is marked underdiagnosis and undertreatment of cardiac risk in RA. Several major barriers exist, including lack of time. Most rheumatologists feel that this aspect of care is the responsibility of primary care physicians.

Checkpoint inhibitor-associated autoimmunity.

Checkpoint inhibitors (CI) have revolutionized the management of many cancers but can result in immune-related adverse events (IRAE). In this chapter, we review the clinical manifestations and management of the most common IRAE, plus less common IRAE, such as inflammatory arthritis, of particular interest to rheumatologists. We review the mechanism of action of CI, predictors of IRAE, and the impact of IRAE on cancer outcomes. The study of IRAE is in its infancy; there are very few prospective studies and virtually no treatment trials. Where possible, we have drawn estimates of IRAE incidence from meta-analyses of randomized controlled trials. Clinical descriptions are derived from case series and case reports. Readers are encouraged to refer to consensus guidelines for IRAE management published by the American Society of Clinical Oncology and the Society for Immunotherapy of Cancer.

A review of the effects of statins in systemic sclerosis.

OBJECTIVES: We performed a literature review assessing possible benefits of statins in systemic sclerosis (SSc). METHODS: PubMed, Embase, Cochrane Databases, and Medline were searched. Full-text English publications were identified in which the effects of statins in SSc were examined. Letters, review articles, and studies on morphea were excluded. RESULTS: In all, 18 of 404 studies were relevant. In vitro, statins decreased transcription and translation of IL-6 and collagen, with reversal via mevalonate. Animal studies demonstrated reduced production of Ras (a protein superfamily of GTPases), Rho (part of the Ras superfamily), and extracellular signal-regulated kinases (ERK), less fibrosis and myofibroblast transdifferentiation, and improved macrovasculature. In human studies, IL-6, an inflammatory cytokine, was reduced. Usually endothelial progenitor cell concentrations increased, and flow-mediated dilatation improved. Raynaud's phenomenon, digital ulcers, and physician global assessments improved in the majority of studies of statin treatment in SSc. None of the 256 patients receiving statins experienced transaminitis or myopathy. CONCLUSIONS: Not all findings were consistent. However, in general, in vitro, animal, and human studies demonstrated benefit in SSc pathophysiology, likely mediated through inhibition of lipid intermediate synthesis. Clinical improvement in SSc circulatory complications was observed. Statins seemed safe and well tolerated in SSc. Larger longer-term multi-site randomized trials are needed to further determine the role of statins as adjunctive treatment of this complex, heterogeneous connective tissue disease.

Factors Associated with Low Flow in Aortic Valve Stenosis.

BACKGROUND: Low-flow low-gradient aortic stenosis (AS) is a predictor of worse outcome compared with normal-flow AS. Although depressed left ventricular ejection fraction (LVEF) is associated with low flow, there is less evidence to support the role of other indices of cardiac structure and function. METHODS: Clinical and echocardiographic data from patients with native AS and valve areas ≤ 1.0 cm(2) were retrospectively analyzed to identify characteristics that are associated with low-flow low-gradient AS. RESULTS: In total, 941 patients were included. On multivariate analysis, factors independently associated with low flow (stroke volume index < 35 mL/m(2)) included worse right ventricular systolic function, atrial fibrillation, lower LVEF, and higher left ventricular mass, with moderate or severe mitral regurgitation independently associated with low flow in the 694 patients (74%) with preserved LVEFs. CONCLUSIONS: Right ventricular dysfunction and atrial fibrillation are independently associated with low-flow low-gradient AS, while moderate or severe MR is independently associated with low flow in patients with preserved LVEF. These associations with low flow in AS are clinically important to recognize, to avoid underestimation of AS severity.

Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the face: An unusual presentation.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare entity that usually occurs as 0.5-3 cm pink to brown nodules in the skin surface and subcutaneous tissues of the head & neck. Here we report an unusual occurrence in size and appearance of ALHE in the tissues of the cheek of an adult Asian male.