PMID- 15242489
OWN - NLM
STAT- MEDLINE
DCOM- 20050315
LR  - 20061115
IS  - 0815-9319 (Print)
IS  - 0815-9319 (Linking)
VI  - 19
IP  - 8
DP  - 2004 Aug
TI  - Prevalence of immune disturbances and chronic liver disease in family members of 
      patients with primary biliary cirrhosis.
PG  - 873-8
AB  - BACKGROUND AND AIMS: Primary biliary cirrhosis (PBC) has been reported in up to 
      4-6% of first degree relatives of patients with the disease. In addition, immune 
      abnormalities, including hypergammaglobulinemia, autoantibodies and increased 
      frequency of autoimmune disorders, were reported in family members of PBC 
      patients. The aim of the present study was to investigate the prevalence of PBC 
      in relatives of patients with PBC, and to investigate the occurrence of chronic 
      liver disease (CLD) and immune abnormalities in these subjects. METHODS: 
      One-hundred first degree relatives of 26 patients with PBC were interviewed and 
      submitted to physical examination and determination of liver enzymes, 
      gamma-globulin, bilirubin and auto-antibodies, including antinuclear (ANA), 
      antismooth muscle (SMA), antimitochondrial antibodies (AMA) by indirect 
      immunofluorescence (IIF) and anti-M2 antibody by immunoblotting (IB). RESULTS: 
      Immune disturbances were rarely observed in relatives of PBC patients. Higher 
      gamma-globulin levels, SMA and ANA were detected in four, eight and two family 
      members, respectively. In most subjects, these autoantibodies were either in low 
      titers or associated with concurrent diseases. Only four relatives had 
      extrahepatic autoimmune diseases and another eight exhibited other CLD. Primary 
      biliary cirrhosis was detected in a sister of one patient. Additionally, two 
      other relatives of PBC patients who tested negative for AMA by IIF showed 
      reactivity for anti-M2 by IB. CONCLUSIONS: Immune disturbances, including ANA and 
      SMA, are uncommon in family members of PBC patients. Conversely, anti-M2 
      antibodies and overt PBC do occur in relatives of PBC patients, even in Brazil 
      where the disease is quite rare.
FAU - Bittencourt, Paulo Lisboa
AU  - Bittencourt PL
AD  - Department of Gastroenterology, University of Sao Paulo School of Medicine, Sao 
      Paulo, Brazil. plbbr@uol.com.br
FAU - Farias, Alberto Queiroz
AU  - Farias AQ
FAU - Abrantes-Lemos, Clarice Pires
AU  - Abrantes-Lemos CP
FAU - Goncalves, Luciana Lofego
AU  - Goncalves LL
FAU - Goncalves, Patricia Lofego
AU  - Goncalves PL
FAU - Magalhaes, Emilia Pereira
AU  - Magalhaes EP
FAU - Carrilho, Flair Jose
AU  - Carrilho FJ
FAU - Laudanna, Antonio Atilio
AU  - Laudanna AA
FAU - Cancado, Eduardo Luiz Rachid
AU  - Cancado EL
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Australia
TA  - J Gastroenterol Hepatol
JT  - Journal of gastroenterology and hepatology
JID - 8607909
RN  - 0 (Autoantibodies)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Autoantibodies/blood
MH  - Autoimmune Diseases/*epidemiology/immunology
MH  - Chronic Disease
MH  - Family
MH  - Female
MH  - Humans
MH  - Immunologic Tests
MH  - Liver Cirrhosis, Biliary/*epidemiology/immunology
MH  - Liver Diseases/*epidemiology/immunology
MH  - Liver Function Tests
MH  - Male
MH  - Middle Aged
MH  - Prevalence
EDAT- 2004/07/10 05:00
MHDA- 2005/03/16 09:00
CRDT- 2004/07/10 05:00
PHST- 2004/07/10 05:00 [pubmed]
PHST- 2005/03/16 09:00 [medline]
PHST- 2004/07/10 05:00 [entrez]
AID - JGH3396 [pii]
AID - 10.1111/j.1440-1746.2004.03396.x [doi]
PST - ppublish
SO  - J Gastroenterol Hepatol. 2004 Aug;19(8):873-8. doi: 
      10.1111/j.1440-1746.2004.03396.x.