The American journal of the medical sciences
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Review Case Reports
Multiple fibrotic lung nodules in a patient with primary Sjögren's syndrome.
Evaluation of bilateral lung nodules noted on imaging poses a diagnostic challenge to clinicians as it can have many differentials from benign to malignant causes. It becomes especially critical to identify them right when there are underlying autoimmune conditions and risk factors for infection. However, a thorough investigation can lead to the recognition of rare associations as described below. ⋯ This lead to the suspicion that these nodules could be related to underlying Sjögren's syndrome. Initial inpatient management with intravenous steroids showed significant improvement in her symptomatology. Hence, we present this rare association of lung nodules with Sjögren's syndrome and its management for awareness of this condition.
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Review Case Reports
Lung recovery with prolonged ECMO following fibrotic COVID-19 acute respiratory distress syndrome.
The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus has been associated with acute respiratory distress syndrome (ARDS) and in some cases with pulmonary fibrosis. There is limited information regarding the long-term outcomes of patients who develop severe COVID-19 infection and subsequent pulmonary fibrosis. ⋯ Further, our patient developed severe parenchymal and airway distortion but ultimately resolved pulmonary fibrosis many months into the hospitalization. In addition to our detailed case discussion, we will provide a focused review on pulmonary fibrosis post COVID-19.
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Xanthogranulomatous pyelonephritis (XGP) is a rare variant of chronic pyelonephritis, occurring in the setting of obstructive uropathy and recurrent urinary tract infections (UTIs). It is difficult to diagnose as it can be asymptomatic until late-stage disease. Localized symptoms such as flank pain and dysuria may be attributed to nephrolithiasis or UTIs without prompting need for further workup. ⋯ A delay in diagnosis can lead to fulminant complications or a more technically difficult nephrectomy. We present three cases of XGP, which serve to highlight the possibility of earlier diagnosis and resultant management options, including the potential for nephron-saving strategies. Early clinical and radiologic suspicion through awareness of risk factors may play an important role in preventing disease progression, avoiding late-stage complications, and improving treatment outcomes.
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The term "subpleural sparing" refers to computed tomography (CT) images that indicate that there is limited disease/infiltrate in the immediate subpleural location. This observation is often associated with nonspecific interstitial pneumonitis and is a characteristic that distinguishes this pathology from usual interstitial pneumonitis (idiopathic pulmonary fibrosis). Subpleural sparing can also occur in acute respiratory disorders, including pulmonary contusion in children, acute lung disease associated with electronic cigarettes (vaping), and aspiration of exogenous lipids. ⋯ Consequently, the density and distribution of lymphatics could explain preferential clearing of the subpleural regions during acute injury. The acquisition of CT images also depends on the configuration of detectors, slice thickness, and the energy of the electron beam. Clinicians should carefully consider the disease process, lymphatic function and other clearance mechanisms, and the vagaries in CT image acquisition when they evaluate patients with subpleural sparing.
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Review Case Reports
What lies in-between: C3 glomerulopathy with non-hemolytic renal microangiopathy and an ultra-rare C3 variant.
We report a 36-year-old female with mixed nephritic-nephrotic syndrome and recurrent pancreatitis. Kidney biopsy showed a crescentic membranoproliferative glomerulonephritis with dominant C3 staining on immunofluorescence (IF) but only scant deposits on electron microscopy (EM) and instead, evidence of severe acute and chronic microangiopathy - endothelial swelling, sub-endothelial fluff, and segmental basement membrane remodeling. Her serum C3 was normal, Factor Ba, and serum Membrane attack complex (sMAC) levels were elevated, and Properdin was low. ⋯ She showed an initial response to terminal complement blockade with eculizumab, but her renal disease progressed in the next year. Notably, our patient never demonstrated microangiopathic hemolysis, yet pancreatitis of unclear etiology recurred periodically. Our case suggests the existence of a "C3G/aHUS overlap" clinicopathologic syndrome and highlights the challenges of treating complement-mediated kidney disease.