The American journal of the medical sciences
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Review Case Reports
Chronic Dyspnea with Raynaud's Phenomenon and Elevated ANA: A Diagnosis of Systemic Sclerosis Sine Scleroderma.
Systemic sclerosis (SSc) should be considered in all patients initially diagnosed with idiopathic interstitial lung disease (ILD), even in the absence of classical scleroderma cutaneous features. Systemic sclerosis sine scleroderma (ssSSc) is a rare subtype of SSc, and the diagnosis requires the absence of characteristic skin thickening but the presence of the three following criteria: (A) Raynaud's phenomenon or the equivalent of abnormal nail fold capillaries, (B) positive antinuclear antibody (ANA), typically with nucleolar or speckled immunofluorescence pattern, and (C) at least one internal organ involvement of ILD, renal dysfunction, esophageal/bowel dysmotility or pulmonary arterial hypertension; in the absence of an alternative rheumatological diagnosis. The radiological and histopathological features of systemic sclerosis sine scleroderma-associated interstitial lung disease (ssSSc-ILD) are commonly those of non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) that cannot help distinguish between idiopathic interstitial pneumonia, different types of connective tissue diseases, or even different subsets of SSc. ⋯ We present a case of a 58-year-old woman presenting with chronic dyspnea, a positive review of systems for Raynaud's phenomenon, and found to have elevated nucleolar immunofluorescence pattern of ANA with chest imaging consistent with the diagnosis of ssSSc-ILD. The uniqueness of this case is that despite symptomatic alleviation with oral mycophenolate therapy, our patient's restrictive lung disease on pulmonary function tests continued to decline, requiring initiation of oral nintedanib therapy leading to stability and improvement. However, due to the rarity of ssSSc, the use of oral nintedanib for systemic sclerosis-associated ILD has only been formally assessed on patients with diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis.
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Liver transplant (LT) is a lifesaving treatment for patients with end stage liver disease. Historically, institutions across the United States have deemed active marijuana use as an exclusion criterion for listing. This study aims to investigate LT outcomes in patients with history of marijuana use prior to LT. ⋯ Our data indicates that marijuana is not associated with increased risk of postoperative noncompliance, other organ complications, infections, or death. As a single factor, marijuana may not need to be a contraindication for LT.
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Ankle brachial index (ABI) as a risk-enhancing factor in addition to the pooled cohort equation (PCE) in assessing cardiovascular risk for primary prevention of atherosclerotic cardiovascular disease (ASCVD) is uncertain. ⋯ Using the PCE, two-third of the participants with low ABI were classified as having a low, borderline or intermediate risk of ASCVD. Low ABI was associated with an increased all-cause mortality in the overall cohort and specifically among those with a borderline/intermediate or high risk of ASCVD but not in those with a low risk of ASCVD. Our study supports consideration of ABI as a risk enhancer for primary prevention among patients classified as borderline or intermediate risk of ASCVD.