Inflammatory bowel disease (IBD) is related to different liver extraintestinal manifestations and occurs with or without a link to disease activity. Primary sclerosing cholangitis (PSC) is the most common hepatobiliary manifestation. Other autoimmune hepatopathies may develop during the evolution of the latter, which is known as overlap syndrome. Sequential overlap syndrome occurs when these conditions appear in subsequent stages, and it is less frequently associated with IBD. We report three cases of sequential overlap syndrome with autoimmune hepatitis as the first manifestation, followed by PSC after 7-19 years and subsequently IBD. Liver extraintestinal manifestations may precede IBD by several years. Therefore, it is crucial to keep this association in mind, thereby reducing the diagnostic delay.
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http://dx.doi.org/10.17235/reed.2020.6938/2020 | DOI Listing |
Background: When haemolytic anaemia, thrombocytopenia and renal failure are present, a thrombotic microangiopathic (TMA) condition should be suspected. We describe the various differential diagnoses of primary TMA syndromes, their clinical findings, clinical workup and treatment.
Case Presentation: A previously healthy man in his fifties was hospitalised with anaemia, thrombocytopenia, bilirubinaemia and acute renal failure.
Cureus
December 2024
General Practice, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, BRA.
Felty's syndrome (FS) is a rare and complex condition most commonly seen as a complication of longstanding rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly, and neutropenia. Diagnosing FS can be challenging due to its diverse clinical presentations and overlap with other hematologic and autoimmune conditions. We report a 47-year-old male with a history of severe anemia, recurrent blood transfusions, and a chronic leg ulcer.
View Article and Find Full Text PDFBr J Hosp Med (Lond)
December 2024
Department of Rheumatology, University College London (UCL) Hospital Foundation Trust, London, UK.
Sjögren disease (SjD) is a systemic autoimmune disorder affecting both children and adults, with a wide range of clinical phenotypes. It remains a challenging condition to recognise and diagnose early and manage effectively. The heterogeneous nature of the presentation, variable disease course and overlapping symptoms with other autoimmune conditions often result in delayed diagnosis.
View Article and Find Full Text PDFThe disproportionate risk for idiopathic proteinuric podocytopathies in Black people is explained, in part, by the presence of two risk alleles (G1 or G2) in the gene. The pathogenic mechanisms responsible for this genetic association remain incompletely understood. We analyzed glomerular RNASeq transcriptomes from patients with idiopathic nephrotic syndrome of which 72 had inferred African ancestry (AA) and 152 did not (noAA).
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