https://www.selleckchem.com/products/blasticidin-s-hcl.html A 57-year-old man was referred to our department because of progressive shortness of breath and emaciation. He had experienced pneumothorax three times in the past five years. The patient radiologically showed mild upper-lobe predominant airspace consolidation and severe platythorax and was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis (PPFE). Although the wedge-shaped shadows in the bilateral lung apexes did not significantly progress, his platythorax gradually worsened during the clinical course. He ultimately died of chronic respiratory failure 1.2 years after the diagnosis. This case demonstrates a rare variant of idiopathic PPFE with progressive platythorax disproportionate to the extent of upper-lobe fibroelastosis.The etiology of Sjögren's syndrome (SS) remains unclear and is associated with many other autoimmune diseases. In particular, symptoms of SS are not apparent when steroids are already being administered for other autoimmune diseases. This report documents a case of autoimmune hepatitis with SS, which was diagnosed on the basis of the worsening of unilateral pleural effusion after the discontinuation of steroids as well as the manifestation of symptoms, such as dry mouth. Adrenal insufficiency due to the discontinuation of steroids was assumed to be the cause of the worsening, rather than infection stress, because no indicators of infectious diseases were observed. The diagnosis of SS was confirmed via lip biopsy examination and anti-SS antibody positivity. Re-administration of steroids rather than antibiotics drastically reduced the pleural effusion and improved the dry mouth symptom. SS with pleural effusion in a case of autoimmune disease was reported to show both unilateral and bilateral pleural effusion predominantly containing lymphocytes. SS with pleural effusion may be more common than expected and should be differentiated from traditional SS. Moreover, biopsy