scholarly journals Chronic recurrent multifocal osteomyelitis and primary sclerosing cholangitis with type 1 autoimmune hepatitis in a child with ulcerative colitis: a case report

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Hon Yan Ng ◽  
Orlee R. Guttman ◽  
Lori B. Tucker
Keyword(s):  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Unique Case ◽  
Case Presentation ◽  
Multifocal Osteomyelitis ◽  
Inflammatory Bowel ◽  
Bone Inflammation

Abstract Background Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a condition characterized by sterile bone inflammation, usually occurring in childhood. Although the etiology remains unclear, this condition has been associated with inflammatory bowel disease (IBD). Primary sclerosing cholangitis (PSC) and Autoimmune Hepatitis (AIH) are also uncommon pediatric conditions with a known association with IBD. Case presentation We present a unique case of a pediatric patient with an initial diagnosis of CRMO, with subsequent diagnosis of autoimmune hepatitis and PSC overlap, and eventually IBD. Conclusions Patients with CRMO may also develop PSC in addition to IBD, further highlighting the importance of IBD pathophysiology in both conditions. Clinical screening of associated gastrointestinal findings may be of value in patients with CRMO.

Difficulties in differential diagnosis of inflammatory bowel diseases in patient with overlapping autoimmune hepatitis and primary sclerosing cholangitis

2021 ◽  
Vol 1 (6) ◽  
pp. 26-30
Author(s):  
T. S. Krolevets ◽  
M. A. Livzan ◽  
M. I. Plinder
Keyword(s):  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Accurate Diagnosis ◽  
Eosinophilic Colitis ◽  
Morphological Picture ◽  
Bowel Diseases ◽  
Inflammatory Bowel ◽  
Autoimmune Hepatitis Type 1

The article presents a clinical case of cross-syndrome of autoimmune hepatitis type 1 with primary sclerosing cholangitis. The main symptoms, laboratory and instrumental criteria of these diseases are discussed. Detection of specific antibodies is necessary for an accurate diagnosis. Interpretation of changes in the colon obtained by stepwise biopsy requires special attention. The described morphological picture does not allow to make an accurate diagnosis, it can be regarded as initial manifestations of Crohn's disease, microscopic or eosinophilic colitis, signs of worm-parasitic invasion. An example of a differential diagnostic search in such a situation is discussed.

Su1984 CLINICAL PRESENTATION AND OUTCOMES OF PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE HEPATITIS IN INFLAMMATORY BOWEL DISEASE

2020 ◽  
Vol 158 (6) ◽  
pp. S-732-S-733
Author(s):  
Camilla A. Martins ◽  
Ana Elisa R. Caon ◽  
Marilia G. Cruz ◽  
Luísa L. Barros ◽  
Alexandre Carlos ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Bowel Disease ◽  
Sclerosing Cholangitis ◽  
Clinical Presentation ◽  
Inflammatory Bowel

Autoimmune Hepatitis/Overlap Syndromes

2018 ◽  
Author(s):  
Albert J Czaja
Keyword(s):  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Clinical Evidence ◽  
Calcineurin Inhibitors ◽  
Primary Biliary Cholangitis ◽  
Histological Findings ◽  
Overlap Syndromes ◽  
Inflammatory Bowel ◽  
Lymphoplasmacytic Infiltration

When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.  This review contains 6 figures, 7 tables and 50 references Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features

scholarly journals The Evolution of Very Early Onset Inflammatory Bowel Disease, Autoimmune Hepatitis, and Primary Sclerosing Cholangitis in a Young Girl

2021 ◽  
pp. 939-947
Author(s):  
Aditi Dey ◽  
Jacquelin Peck ◽  
Michael Wilsey ◽  
Jolan Walter ◽  
Johnny Nguyen ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Bowel Disease ◽  
Early Onset ◽  
Sclerosing Cholangitis ◽  
Autoimmune Disorders ◽  
Feeding Difficulties ◽  
Gastrointestinal Problems ◽  
Inflammatory Bowel

Very early onset inflammatory bowel disease, autoimmune hepatitis (AIH), or primary sclerosing cholangitis (PSC) alone is a rare condition in young children. The combination of all 3 autoimmune disorders in a 16-month-old child is even rarer. The onset and etiology of these diseases is multifactorial and typically unknown. However, when the children are diagnosed, the accepted view point is that the inflammation was likely present for months to years prior. This case is unique because the gastrointestinal problems started from infancy, and evolved to the development of Crohn’s disease, AIH, and PSC at a very early age. This case helps bring to light that very early onset autoimmune disorders may in fact present with symptoms of feeding difficulties, growth failure, and formula intolerance. Patients may be diagnosed initially with allergic enterocolitis in infancy. Although few children with these symptoms evolve to develop autoimmune diseases at an older age, clinicians should consider following these children more closely. This case also demonstrates how hard it is to diagnose very early onset autoimmune disorders, as they mimic other illnesses.

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