Internal medicine
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A 42-year-old woman presented with muscle weakness and hypokalemic distal renal tubular acidosis (dRTA). Investigations revealed concurrent Sjögren's syndrome (SS) and Hashimoto's thyroiditis contributing to hypokalemic dRTA. ⋯ Rapid improvement in tubular injury markers and hypobicarbonemia followed potassium supplementation, suggesting that hypokalemia contributed to proximal tubular injury. This case underscores the diagnostic challenge posed by the simultaneous presence of TIN and hypokalemic nephropathy, potentially masking hypokalemic nephropathy in patients with hypokalemic dRTA secondary to SS-TIN.
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Objective Patients with advanced interstitial lung disease (ILD) struggle to undergo spirometry to evaluate the respiratory function. The cardiothoracic ratio (CTR) on chest radiography can potentially reflect the lung volume; however, this has not yet been fully established. This study aimed to clarify the relationship between the CTR and the respiratory function in patients with interstitial lung diseases. ⋯ The CTR was significantly negatively correlated with the vital capacity (r = -0.490, p <0.001). Conclusions An increased CTR might reflect a decreased vital capacity, but not a decreased cardiac function, in patients with interstitial lung diseases. Measuring the CTR can thus be beneficial for predicting progression in patients with ILD.
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A 64-year-old woman presented with agranulocytosis, anemia, and bacteremia, leading to a diagnosis of T-cell large granular lymphocytic leukemia (T-LGLL). A molecular analysis identified a signal transducer and activator of transcription 3 (STAT3) Y640F variant. ⋯ The patient underwent cord blood transplantation (CBT) after preconditioning with fludarabine, busulfan, and total body irradiation, yielding a STAT3 Y640F variant disappearance, based on allele-specific quantitative polymerase chain reaction (AS-qPCR). In this case, CBT is a promising refractory T-LGLL treatment option, and the STAT3 Y640F variant AS-qPCR is a T-LGLL activity marker.
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A 58-year-old woman was admitted to our hospital with anasarca and generalized lymphadenopathy. Laboratory data showed serum Cr 1.48 mg/dL, CRP 2.38 mg/dL, PLT 102,000/μL, and anti-SS-A antibodies (SS-A-ab). ⋯ Her body weight decreased from 59.4 to 41 kg, and pleural effusion disappeared 8 weeks later. This case suggests that TAFRO-like symptoms may occur in patients with Sjögren's syndrome with HTLV-1 infection.
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Macrophage activation syndrome (MAS) involves an excessive amount of acute inflammatory responses to inflammatory cytokines, particularly interleukin-6 (IL-6). IL-6 is also strongly associated with the pathophysiology of certain neuroimmunological diseases. ⋯ Standard immunosuppressive therapies could not stabilize the symptoms in our cases until antibodies against the IL-6 receptor were administered. This finding suggests that it is important to consider the underlying pathophysiology of MAS in relation to these neuroimmunological diseases when treating affected patients.