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Breaking down the condition of cryptogenic cirrhosis

DEAR DR. ROACH: My friend has cryptogenic cirrhosis. She says that the doctors don’t know what causes it. Where could this have come from, and what does this mean for her? — A.T.G.

ANSWER: “Cryptogenic” is from the Greek roots “krytpo-” (meaning hidden) and -genic (for “origin”), so “cryptogenic” literally means that its origins are unknown. Cirrhosis is end-stage liver disease, which can come from many different causes such as alcoholic liver disease, viral hepatitis, or metabolic liver disease due to fat deposition in the liver.

The term is used for people with cirrhosis where the cause is not clear. It is thought that many cases of cryptogenic cirrhosis are due to metabolic-associated steatotic liver disease (MASLD). Once a person has progressed to cirrhosis, the inflammation and fat can sometimes no longer be identified in the liver biopsy specimen. A past history of being overweight or having diabetes makes MASLD more likely.

Other known causes of cryptogenic cirrhosis include hepatitis viruses. Before we had a reliable blood test for hepatitis C, it was a common cause of what was then cryptogenic cirrhosis. It is possible there may be other viruses that cause cirrhosis and are, as yet, uncharacterized. Autoimmune hepatitis can lead to cirrhosis and might not be identified. Rare genetic mutations (keratin protein, short telomere syndrome) can also be the underlying cause.

Once a person has cirrhosis, there is generally no cure except liver transplantation. All cases of cirrhosis predispose a person to liver cancer, so a person with cryptogenic cirrhosis will be recommended for screening, usually consisting of blood testing and ultrasounds. Alcohol must be avoided, and lifestyle changes to help metabolic liver disease is often recommended, since this is a presumed cause of many cases of cryptogenic cirrhosis.

About 50% of people with cryptogenic cirrhosis will be alive for five years after their diagnosis. Ideally, a person will have a primary care doctor, a liver specialist, and a transplant team working together to help treat their cryptogenic cirrhosis, including preventive maneuvers such as a vaccination for viral hepatitis, which can be rapidly fatal in a person with cirrhosis from any cause. People with cirrhosis of any kind should avoid raw shellfish.

DEAR DR. ROACH: I have occasionally gotten spells of zig-zag shapes and blind spots in my vision. After a few of these, I found that if I drank a pint of water and waited 10 minutes, I would be just fine. I heard from a friend who had one of these spells, and it really frightened her. If this is truly a sign of dehydration, perhaps others who experience this might benefit from knowing this. — J.F.

ANSWER: The sudden onset of blind spots in the vision is potentially a medical emergency; this should always be taken seriously, as it can signify a stroke.

However, when people not only lose their vision but also have things in their vision that don’t belong there, like bright lights or the zig-zag lines you mention, it is highly suggestive of a migraine aura without headache. Most people with this have also had a migraine headache, but some people only get the aura and never have the headache. These people are almost always over 50 and are at risk for stroke, so it’s very important to evaluate this potential warning sign promptly.

When a person has had the same type of symptoms several times that get better within an hour (I’m not sure if the water helped, or if it would have gone away on its own), it’s very likely to be a migraine aura without headache.

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