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Association Between Sjogren Syndrome, Primary Biliary Cirrhosis and Interstitial Lung Disease
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A6533 - Association Between Sjogren Syndrome, Primary Biliary Cirrhosis and Interstitial Lung Disease
Author Block: A. Das1, A. Faisal1, A. Kumar2; 1Internal medicine, Spectrum health, Grand Rapids, MI, United States, 2Pulmonary and Critical Care, Spectrum health -Michigan State University College of Human medicine, Grand Rapids, MI, United States.
68 year old female smoker,presented with gradual onset weakness and falls.She also reported increased shortness of breath.Past medical history was significant for Primary biliary cirrhosis.Vital signs were unremarkable except for new onset hypoxia. Physical examination showed bibasilar crackles and diffuse neuromuscular weakness. CT scan chest showed prominent areas of parenchymal change within the region of the left upper lobe and lingula. Severe conglomerate areas of fibrotic change or infiltrates were noted in the lower lobes. Prominent mediastinal and hilar lymphadenopathy was noted. Serology worked up showed positive ANA and positive SSA/B.
Primary biliary cirrhosis is associated with underlying autoimmune diseases. The most common auto-immune disease associated with PBC was Sjogren syndrome (SS)(36.2%).Conversely,clinically overt PBC was found in less than 2% SS patients in large cohorts.Concomitant SS increases the risk of ILD in patients with PBC.
Author Block: A. Das1, A. Faisal1, A. Kumar2; 1Internal medicine, Spectrum health, Grand Rapids, MI, United States, 2Pulmonary and Critical Care, Spectrum health -Michigan State University College of Human medicine, Grand Rapids, MI, United States.
68 year old female smoker,presented with gradual onset weakness and falls.She also reported increased shortness of breath.Past medical history was significant for Primary biliary cirrhosis.Vital signs were unremarkable except for new onset hypoxia. Physical examination showed bibasilar crackles and diffuse neuromuscular weakness. CT scan chest showed prominent areas of parenchymal change within the region of the left upper lobe and lingula. Severe conglomerate areas of fibrotic change or infiltrates were noted in the lower lobes. Prominent mediastinal and hilar lymphadenopathy was noted. Serology worked up showed positive ANA and positive SSA/B.
Primary biliary cirrhosis is associated with underlying autoimmune diseases. The most common auto-immune disease associated with PBC was Sjogren syndrome (SS)(36.2%).Conversely,clinically overt PBC was found in less than 2% SS patients in large cohorts.Concomitant SS increases the risk of ILD in patients with PBC.
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