Abstract
Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti-smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression.
| Original language | English |
|---|---|
| Pages (from-to) | 1135-1144 |
| Number of pages | 10 |
| Journal | Hepatology |
| Volume | 43 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - May 2006 |
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ASJC Scopus subject areas
- Hepatology
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Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis. / Wesierska-Gadek, Józefa; Penner, Edward; Battezzati, Pier Maria; Selmi, Carlo; Zuin, Massimo; Hitchman, Eva; Worman, Howard J.; Gershwin, M. Eric; Podda, Mauro; Invernizzi, Pietro.
In: Hepatology, Vol. 43, No. 5, 05.2006, p. 1135-1144.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis
AU - Wesierska-Gadek, Józefa
AU - Penner, Edward
AU - Battezzati, Pier Maria
AU - Selmi, Carlo
AU - Zuin, Massimo
AU - Hitchman, Eva
AU - Worman, Howard J.
AU - Gershwin, M. Eric
AU - Podda, Mauro
AU - Invernizzi, Pietro
PY - 2006/5
Y1 - 2006/5
N2 - Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti-smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression.
AB - Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti-smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression.
UR - http://www.scopus.com/inward/record.url?scp=33646571925&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33646571925&partnerID=8YFLogxK
U2 - 10.1002/hep.21172
DO - 10.1002/hep.21172
M3 - Article
C2 - 16628641
AN - SCOPUS:33646571925
VL - 43
SP - 1135
EP - 1144
JO - Hepatology
JF - Hepatology
SN - 0270-9139
IS - 5
ER -