Related Experiment Videos
Primary biliary cirrhosis accompanied by CREST syndrome
Gregory Kouraklis1, Andromahi Glinavou, Gabriel Karatzas
1Second Department of Propedeutic Surgery, Medical School University of Athens, Greece.
Southern Medical Journal
|October 3, 2002
Summary
This study reports the first known case of CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) associated with primary biliary cirrhosis (PBC) in a male patient. This rare association highlights the need for clinical awareness in diagnosing both conditions.
Area of Science:
- Rheumatology and Gastroenterology
- Autoimmune and Connective Tissue Diseases
Background:
- CREST syndrome is a variant of systemic scleroderma characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.
- Primary biliary cirrhosis (PBC) is a chronic liver disease, and its association with CREST syndrome is established but primarily documented in women.
Observation:
- A rare case of a male patient presenting with CREST syndrome is described.
- The patient exhibited symptoms of acute cholecystitis and mild jaundice, indicative of PBC.
- This presentation marks the first reported instance of this dual diagnosis in a male individual.
Findings:
- The study confirms a concurrent diagnosis of CREST syndrome and primary biliary cirrhosis in a male patient.
- The clinical manifestations in this case included acute cholecystitis and mild jaundice, linked to PBC.
- This case challenges the previously observed gender prevalence in the association between CREST syndrome and PBC.
Implications:
- Highlights the importance of recognizing the association between CREST syndrome and PBC in male patients.
- Suggests that clinicians should consider CREST syndrome in men presenting with symptoms suggestive of PBC, even if mild.
- Emphasizes the need for increased awareness among healthcare professionals regarding this rare comorbidity to ensure timely diagnosis and management.