My last post was a personal account of my ongoing experience with primary biliary cirrhosis (PBC), and I’ll be offering more of that, but first I need to explain a bit about this condition. I have yet to encounter someone outside the medical world who knows about it, although “PBC is considered to be an uncommon disease, but not rare,” according to MedicineNet, the source I will most rely on here.
Of course there are a number of sites like MedicineNet which have been written and reviewed by the medical profession. This is no substitute for those by any means. But I’ve had some experience with the kinds of questions that people who are not worried about personally being affected ask first, while the health sites I think are usually written for people who have been diagnosed and need more systematically organized info.
First, the name. The unfortunate name.
The primary is OK — it is primary because not secondary or tertiary. It isn’t a result of another condition.
Same goes for biliary: it involves the bile ducts.
As for cirrhosis, it’s unfortunate on two counts.
To begin with, it isn’t entirely accurate. The condition eventually leads to cirrhosis; it isn’t a special variety of cirrhosis.
Secondly, cirrhosis is a loaded word. I bet most people think they know what causes cirrhosis of the liver: chronic alcohol abuse. Some might come up with another cause: hepatitis C (and what causes that? Dirty needles?). And, yes, these are the two most common causes of cirrhosis, and both are, to a greater (chronic alcohol abuse) or lesser (hepatitis C) extent related to choices people make. And we all know that there are those who lack sympathy for alcoholics’ or IV drug users’ health problems.
There are, by the way, a number of other causes of cirrhosis of the liver besides those two, including, in addition to PBC, others that have nothing to do with substance abuse, like haemochromatosis (too much iron in the blood) and Wilson’s Disease (too much copper).
The problem is that
… the name primary biliary cirrhosis is actually a misnomer for patients in the earlier stages of the illness. The more technically correct and ponderous term for PBC, chronic non-suppurative destructive cholangitis. . . has never been widely used and is unlikely to replace PBC.
Chronic non-suppurative destructive cholangitis: I’ve got to agree, that’s not likely to catch on, is it?
So what does cause PBC?
Most of the introductions I’ve read say it is an autoimmune condition, but MedicineNet is a little more cautious in answering the question:
The cause of PBC remains unclear. Current information suggests the cause may involve autoimmunity, infection, or genetic (hereditary) predisposition, acting either alone or in some combination.
. . . PBC is presumed by most experts to be an autoimmune disease, which is an illness that occurs when the body’s tissues are attacked by its own immune (defense) system. (Auto means self.)
. . . Despite strong evidence to support the concept that PBC likewise is an autoimmune disease, some features of PBC are uncharacteristic of autoimmunity. For example, all other autoimmune diseases occur in both children and adults, while, as already mentioned, PBC has never been diagnosed in childhood.
Next time: Who gets it