Monday, October 19, 2015

Lyme aortitis and aneurysm


Patient with chronic Lyme disease typically present to me with a broad collections of symptoms. For simplification, I like to group Lyme symptoms into 5 general categories: 1) constitution – symptoms like fatigue, low grade fevers and night sweats; 2) musculoskeletal – symptoms like joint pain, swelling etc. 4) neurological symptoms including weakness, numbness, tingling etc.; 4) psychiatric symptoms such as anxiety and depression and 5)  other – “everything and anything else.”

Complaints from the 4 “core” categories seen with the vast majority of chronic Lyme patients. In fact, when such complaints are lacking I look extra hard for a non-Lyme explanation of the problem(s).

Then there is everything else.

A longstanding patient was seen in my office today. He suffers primarily with a profound motor/sensory neuropathy. (His insurance has refused to cover IVIG). He has had cognitive issues and joint issues which have largely resolved. He denies any cardiac symptoms. A recent abdominal ultrasound, performed to rule out gallbladder disease, serendipitously discovered a small thoracic aortic aneurysm. There were no typical risk factors for this. No hypertension, atherosclerosis, family history, Marfan’s syndrome or Ehler’s Danlos syndrome.

He went to a cardiologist who performed an echocardiogram and a significant, aortic root aneurysm, 4 cm was found. The patient asked me if this might be due to Lyme disease. After all, Lyme is like syphilis and syphilis is famously known to cause “luetic aneurysms” of the ascending aorta. I thought not.  I do not think Lyme and syphilis have all that much in common. They come from differing phylogenetic heritage. In general, Lyme is much worse.

Lyme patients tend to have a lot of cardiac symptoms like palpitations and irregular beats including PVCs. They frequently have POTS, but this is a neurological disease, not a primary cardiac one. Lyme patients famously have Lyme carditis causing heart block, an electrical rhythm disturbance frequently requiring a pace maker. Lyme can also directly affect the heart muscle (very rarely) causing a dilated cardiomyopathy and congestive heart failure. Lyme can cause inflammation of the pericardium, the sack around the heart and cause pericarditis. One of my patients who had already been intensively treated for Lyme disease) developed constriction around the heart from pericarditis, called tamponade, requiring emergency surgery to relieve the pressure.

I did not think that Lyme, like syphilis, caused aortic root aneurysms: I was wrong.

In “Pathology, 2014” 300 cases, sections of ascending aortic aneurysms were reviewed. There were 21 cases of aortitis or inflammation of the aorta with 19 aneurysms. Associated causes included: temporal arteritis, ankylosing spondylitis and undifferentiated autoimmune disease; IgA nephropathy, fibromyalgia and Lyme. Fibromyalgia is of course suspect for undiagnosed Lyme.

My patient’s proximal aortic aneurysm is 4 cm and may expand over time – with surgery recommended at 6 cm.
Do I think Lyme patients should be screened for heart disease? An EKG is a good idea since it screens for heart block and occasionally detects other conditions. I do not think screening echocardiograms are warranted. But what doctors call “the index of suspicion” should be low for ordering the test

6 comments:

  1. Nice article. This is so much more than I needed! But will all come in use thanks!
    identify a problem

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  2. Thanks!

    I was bitten in June 2008, got the rings, immediate negative, then 6 weeks later a positive WBlot. In that first year, I think I had more positives even though I'd taken Doxy immediately for a month or so. Since then generally the WBs have been negative but have had debilitating tennis elbow in both arms simultaneously for a little while, debilitating shoulder problems (for a short while). Arthritis, etc - though hard to blame Lyme since it may or may not be the cause I guess (47 y/o male).

    Last week, weird p-wave = sent to Echocardiogram. That shows seemingly normal chambers and valves but a dilated aortic root at 4.2 cm. I had an echo 9 months ago (due to slight shortness of breath) and nothing like that was noted so either it was missed, or has developed since.

    Anyway, that is all I have to say about that. At this time. Except that this sucks.

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  3. Oh - to add - I am going to a cardiologist tomorrow morning so everything is indeterminate right now.

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  4. Considering how many cases of lyme are undiagnosed, it seems prudent to not rule out any symptoms in advance, such as aneurysms. Here a couple of published papers. If it has been found once or twice, there are probably more cases, if anyone looked.

    https://www.ncbi.nlm.nih.gov/pubmed/21842293
    https://www.hindawi.com/journals/scientifica/2014/564734/
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2170070/

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  5. I am a 52 year old man who has been recovering for two months from open heart surgery to repair a 5.3 cm thoracic aortic aneurysm. It was discovered 9 years ago while suffering from a bad case of late-stage Lyme which went untreated for eight months. No one can say for sure if the Lyme caused it, but I have no other risk factors.

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  6. I was also treated for late-stage/chronic Lyme. My symptoms didn't fit perfectly, and they weren't improving with intensive treatment, so I pursued alternate diagnoses at the same time. Slowly an Ehlers Danlos Syndrome diagnosis formed. I was convinced it was vascular, but I still have a Hypermobile-EDS diagnosis to this day due to my genetic screening.

    Despite being examined by several cardiologists over the years, the geneticist sent me specifically for an EDS heart screening. It revealed a 4.5 cm ascending aortic aneurysm, which is the cut-off for open heart surgery for those with EDS/connective tissue disorders. I am their first EDS patient to ever follow this disease course. My recovery was also highly abnormal. The pathology of the aortic wall seemed to indicate consistency with connective tissue disorder though, and my genetic screenings only indicate a mutation on COL12A1. I have an unknown variant, but mutations on that gene are only associated with Myopathic-EDS/Bethlem Myopathy. 6 months after the aortic aneurysm, a small brain aneurysm on my carotid artery was found and is being monitored along with my other major arteries.

    I have no other risk factors, family history/suspicious deaths. Immediate family had echos that came back normal. The genetic aortopathy panel came back normal as well. I am not convinced "Chronic Lyme" is different from "EDS" at this point, as symptom cross-over is significant, and no one doctor has knowledge of both conditions. Perhaps I do have both and it simply magnifies opportunity for extremely rare manifestations. If anyone is capable/interested in testing my aortic wall for Borrelia burgdorferi, my tissue is sitting in a research bank. Just hoping to make it out of my 30's....

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