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Interstitial Lung Disease as Predominant Extrainte ...
Interstitial Lung Disease as Predominant Extraintestinal Manifestation of Ulcerative Colitis
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This case report describes a 48-year-old woman who presented with hematemesis, melena, fever, night sweats, severe dyspnea, and an 80-pound unintentional weight loss over three months. Initial workup showed marked leukocytosis, anemia, elevated inflammatory markers, hypoalbuminemia, and coagulopathy. Chest CT revealed extensive bilateral pulmonary consolidations with cavitary and non-cavitary lesions, raising concern for vasculitis or severe infection. However, bronchoalveolar lavage was negative for infection, and lung biopsy showed organizing pneumonia without vasculitis. Because she failed antibiotics, she was started on prednisone.<br /><br />Further evaluation revealed gastrointestinal and hepatobiliary involvement. Colonoscopy demonstrated diffuse colonic inflammation and friability, especially in the right colon, with rectal and perianal sparing, consistent with ulcerative colitis (UC). Laboratory testing showed positive proteinase-3 antibodies, eosinophilia, and a positive ANA. MRCP and ERCP confirmed primary sclerosing cholangitis (PSC) with multifocal intrahepatic strictures and dominant common bile duct strictures. FibroScan suggested advanced liver fibrosis. IgG4-related disease was ruled out by a low IgG4/IgG ratio, and biopsy showed no dysplasia.<br /><br />The authors emphasize that pulmonary manifestations of inflammatory bowel disease can occur independently of intestinal disease activity and may be the only presenting feature, making diagnosis challenging. Cryptogenic organizing pneumonia is the most common lung manifestation of UC. The report also highlights the association between UC and PSC, which can lead to serious complications such as advanced fibrosis, hepatocellular carcinoma, and cholangiocarcinoma. Early recognition of extra-intestinal manifestations is essential to guide treatment and prevent long-term complications.
Asset Subtitle
Pritee Shrestha
Meta Tag
Author List
Babajide Adio DO, Philip J. Shara DO, Pritee Shrestha
Category
Clinical Vignettes
Concept
Ulcerative Colitis
Concept
Extra-Intestinal Manifestation
Concept
Primary Sclerosing Cholangitis
Concept
Pulmonary Disease
Concept
Secondary Organizing Pneumonia
Distinguished
Non-Finalist
Presenter Organization
Mercy One North Iowa Internal Medicine Residency
Presenting Author
Pritee Shrestha
Track
Adult
Keywords
ulcerative colitis
primary sclerosing cholangitis
cryptogenic organizing pneumonia
pulmonary manifestations
extra-intestinal manifestations
hematemesis
melena
cavitary lung lesions
inflammatory bowel disease
advanced liver fibrosis
Ulcerative Colitis
Extra-Intestinal Manifestation
Primary Sclerosing Cholangitis
Pulmonary Disease
Secondary Organizing Pneumonia
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