From the Journals

CREST syndrome and PBC are often associated


 

FROM THE AMERICAN JOURNAL OF MEDICINE

Primary biliary cholangitis can be associated with limited cutaneous systemic sclerosis (CREST syndrome), according to a clinical communication to the editor from Amirali Kiyani, MD, and Shannon Ursu, MD.

In their case study, a 56-year-old woman presented to the emergency department with a syncopal episode. The patient’s medical history included primary biliary cholangitis (PBC), breast cancer status post lumpectomy and chemoradiation, gastroesophageal reflux disease, Raynaud’s phenomenon, and multiple episodes of gastrointestinal bleeding. Vital signs were normal at the time of admission.

After examination and testing, the patient was found to have low serum complement levels and elevated C-reactive protein. Anti-Scl 70 antibody, SSA, and SSB antibody were negative, and thyroid-stimulating hormone and antitransglutaminase antibodies were normal. No evidence of heart failure or pulmonary hypertension was seen in a transthoracic echocardiogram, and the patient was diagnosed with limited cutaneous systemic sclerosis.

Sjögren’s syndrome and autoimmune thyroiditis are the most common extrahepatic autoimmune disorders associated with PBC, but PBC is associated with CREST syndrome in 1%-6% of cases, according to the literature the investigators reviewed, they said.

“Primary biliary cholangitis is commonly associated with extrahepatic autoimmune disorders such as limited cutaneous systemic sclerosis. … Screening for these autoimmune disorders can prevent further morbidity and keep patients viable candidates for liver transplant,” they concluded.

Find the full clinical communication in the American Journal of Medicine (doi: 10.1016/j.amjmed.2017.05.019).

This story was updated on 9/13/2017.

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