Coxiella burnetii dormancy in a fatal ten-year multisystem dysfunctional illness: case report

[Dolphin]

Contains post-mortem results of somebody you had the diagnosis of “post viral syndrome”

Funding

The authors are grateful to Mrs C. Hunter and Alison Hunter Memorial Foundation in Partnership with the National Centre for Neuroimmunology and Emerging Diseases (NCNED), Griffith University, Queensland, Australia for generous financial support of this investigation.

Free full text:

http://bmcinfectdis.biomedcentral.com/articles/10.1186/s12879-016-1497-z

Coxiella burnetii dormancy in a fatal ten-year multisystem dysfunctional illness: case report.


Sukocheva OA1,2, Manavis J3, Kok TW4, Turra M5, Izzo A6, Blumbergs P3, Marmion BP1,5.


BMC Infect Dis. 2016 Apr 18;16(1):165. doi: 10.1186/s12879-016-1497-z.



Abstract


BACKGROUND:


In a previous study of a Q fever outbreak in Birmingham, our group identified a non-infective complex of Coxiella burnetii (C.b.) antigens able to survive in the host and provoked aberrant humoral and cell-mediated immunity responses.


The study led to recognition of a possible pathogenic link between C.b. infection and subsequent long-term post Q fever fatigue syndrome (QFS).


This report presents an unusually severe case of C.b. antigen and DNA detection in post-mortem specimens from a patient with QFS.


CASE PRESENTATION:


We report a 19-year old female patient who became ill with an acute unexplained febrile encephalitis-like illness, followed by increasingly severe multisystem dysfunction and death 10 years later.


During life, extensive clinical and laboratory investigations from different disciplinary stand points failed to deliver a definitive identification of a cause.


Given the history of susceptibility to infection from birth, acute fever and the diagnosis of "post viral syndrome", tests for infective agents were done starting with C.b. and Legionella pneumophila.


The patient had previously visited farms a number of times.


Comprehensive neuropathological assessment at the time of autopsy had not revealed gross or microscopic abnormalities.


The aim was to extend detailed studies with the post-mortem samples and identify possible factors driving severe disturbance of homeostasis and organ dysfunction exhibited by the course of the patient's ten-year illness.


Immunohistochemistry for C.b. antigen and PCR for DNA were tested on paraffin embedded blocks of autopsy tissues from brain, spleen, liver, lymph nodes (LN), bone marrow (BM), heart and lung. Standard H&E staining of brain sections was unrevealing.


Immuno-staining analysis for astrocyte cytoskeleton proteins using glial fibrillary acidic protein (GFAP) antibodies showed a reactive morphology.


Coxiella antigens were demonstrated in GFAP immuno-positive grey and white matter astrocytes, spleen, liver, heart, BM and LN. PCR analysis

(COM1/IS1111 genes) confirmed the presence of C.b. DNA in heart, lung, spleen, liver & LN, but not in brain or BM.


CONCLUSION:


The study revealed the persistence of C. b. cell components in various organs, including astrocytes of the brain, in a post-infection QFS.


The possible mechanisms and molecular adaptations for this alternative C.b. life style are discussed.


KEYWORDS:
Antigen persistence; Chronic fatigue syndrome; Q Fever

 

[msf]

Well, duh. That´s why the CDC says you need to treat it for 18 months with Doxy.

(I´m saying duh to the authors, not you)

 

[msf]

So, post-Q fever Syndrome is actually chronic Q fever...and post-Lyme Syndrome is...

 

[msf]

Does anyone else think it´s odd that the only chronic bacterial diseases that are treated as such are the ones that kill you fairly quickly? I´m thinking Q fever, Glanders (thanks The Americans!), Tuberculosis. There doesn´t seem to me to be any reason why this should be the case, unless the reason is that it is much harder to ignore people and pretend that they are not really ill when they die at 29.

 

[msf]

´Some of the scepticism and difficulty of acceptance of QFS may have rested on a mistaken assumption that the model of infection with the small intracellular bacterium, Coxiella burnetii, would follow a familiar set of characteristics of invasion, bacteraemia, inflammatory responses, finally resolution with rising antibody levels, acquired cellular immune responses and elimination of the invading organism´

I feel like a lot of doctors make this mistake.

 

[anciendaze]

Coxiella Burnetii is a bacterial pathogen living inside cells, like other Rickettsia. So why was a case of this called a post-viral syndrome?

 

[Scarecrow]

Coxiella Burnetii is a bacterial pathogen living inside cells, like other Rickettsia. So why was a case of this called a post-viral syndrome?

C burnetti wasn't identified until post mortem:

We report a 19-year old female patient who became ill with an acute unexplained febrile encephalitis-like illness, followed by increasingly severe multisystem dysfunction and death 10 years later.

During life, extensive clinical and laboratory investigations from different disciplinary stand points failed to deliver a definitive identification of a cause.

 

[Justin30]

So my guess is that Drs failed to test titers for Q Fever at onset or later on down the line? Using test like in Canada and Europe dont test active infective titers.....they seem to be much more sensitive as many have found by getying tested in the US.

It mentioned that she had Encephalitis like-illness how much do you want to bet the Drs didnt test her spinal fluid.

Labeled her with PVFS and she was left to rot like many people that are labeled with one of a thousand names given to this disease.

I hate reading about these stories...so sad...preventable in my eyes

 

[aaron_c]

Why isn't there a project to do autopsies like this on everyone (they can) who dies with ME/CFS? I know Jered Younger suggested that there are too many confounding factors when someone dies with ME/CFS, but...I don't know if I buy that, as long as you keep everything in perspective.

A second question I have (and I hope someone who knows more can help me) is why we don't do tests like this on people who are alive? I understand that most of us probably don't have Coxiella burnetii, and I also understand that many of the tests done were done on places we don't want to biopsy, like bone marrow or the brain. Finally, I understand that in this case maybe they didn't do the tests in 1996 (which she died) because they weren't available yet. But--and I know very little about testing--why can't we take liver biopsies and test ourselves with PCR tests for...Coxsackie Virus, for example? Or am I talking jibberish?

 

[Dolphin]

Why isn't there a project to do autopsies like this on everyone (they can) who dies with ME/CFS? I know Jered Younger suggested that there are too many confounding factors when someone dies with ME/CFS, but...I don't know if I buy that, as long as you keep everything in perspective.

The ME Association and Action for ME have done a feasibility study on this [post-mortem biobank]. They are interested but don't have the money.