Adult onset still disease associated with endogenous lipoid pneumonia. 2019

Ramin Sami, and Mohammadali Zohal, and Forozan Mohammadi
Department of Internal Medicine, Isfahan University of Medical Sciences, Isfahan, Iran, Isfahan, Iran, Islamic Republic Of.

Cholesterol pneumonia or endogenous lipid pneumonia (ELP) is a rare disease that can occur in the context of a systemic disease or following a bronchial obstruction. It is characterized by a wide range of diverse symptoms and various disease course. The present report introduces a young woman diagnosed with adult onset still disease three years ago, who has been referred with macrophage activation syndrome (MAS). She underwent biopsy due to dyspnea and a crazy paving pattern in HRCT of the lungs, leading to the diagnosis of lipoid pneumonia based on the interstitial lymphocytic inflammation and cholesterol granulomas. So far, there has been no report indicating MAS associated with cholesterol pneumonia. This is the second case reporting ELP in the adult onset still disease.

UI MeSH Term Description Entries
D011014 Pneumonia Infection of the lung often accompanied by inflammation. Experimental Lung Inflammation,Lobar Pneumonia,Lung Inflammation,Pneumonia, Lobar,Pneumonitis,Pulmonary Inflammation,Experimental Lung Inflammations,Inflammation, Experimental Lung,Inflammation, Lung,Inflammation, Pulmonary,Inflammations, Lung,Inflammations, Pulmonary,Lobar Pneumonias,Lung Inflammation, Experimental,Lung Inflammations,Lung Inflammations, Experimental,Pneumonias,Pneumonias, Lobar,Pneumonitides,Pulmonary Inflammations
D002779 Cholestasis Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS). Bile Duct Obstruction,Biliary Stasis,Bile Duct Obstructions,Biliary Stases,Cholestases,Duct Obstruction, Bile,Duct Obstructions, Bile,Obstruction, Bile Duct,Obstructions, Bile Duct,Stases, Biliary,Stasis, Biliary
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001706 Biopsy Removal and pathologic examination of specimens from the living body. Biopsies
D016706 Still's Disease, Adult-Onset Systemic-onset rheumatoid arthritis in adults. It differs from classical rheumatoid arthritis in that it is more often marked by acute febrile onset, and generalized lymphadenopathy and hepatosplenomegaly are more prominent. Adult-Onset Still Disease,Adult-Onset Still's Disease,Still Disease, Adult-Onset,Adult Onset Still Disease,Adult Onset Still's Disease,Adult-Onset Stills Disease,Still Disease, Adult Onset,Still's Disease, Adult Onset,Stills Disease, Adult-Onset
D055501 Macrophage Activation Syndrome A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS. Syndrome, Macrophage Activation

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