C-ANCA Associated vascul-WHAT?!?!

Updated: Mar 8, 2019


C-ANCA associated vasculitis, it's a mouthful, possibly the name of an alien, potentially a universe too?

No, regrettably it's nothing that fancy or that exciting, quite the opposite really.


ANCA associated vasculitis (the bigger umbrella under which subcategories of vasculitis fall) is an Autoimmune disease that causes an immune reaction, which ultimately results in the inflammation of small and medium blood vessels, in other words, vasculitis. There are 3 different subtypes that fall under this umbrella, these include Microscopic Polyangiitis/MPA (my first diagnosis), Granulomatosis with Polyangiitis/GPA, previously known as Wegner's Granulomatosis (my potential diagnosis) and Eosinophilic Granulomatosis with Polyangiitis/EGPA - try stay with me here. As I'm still in the process of being worked up to find which exact type of vasculitis I have, I'll just continue referring to it as C-ANCA associated vasculitis for now.


How on earth does that happen?

Good question. I remember reading about these condition once in medical school, in a textbook on a random day whilst studying about the renal system - the kidneys. Needless to say, it's a pretty rare group of diseases. The international stats show it affects 1-3 per 100 000 people and has an annual incidence of less than 20 per million people (1), so yes, rare indeed. in terms of what causes such a disease, well the verdict is still out on this. The cause is currently unknown, there are many hypotheses that speculate what might be happening but no evidence has been able to hit this nail on it's head.


Immunofluorescence of C-ANCA. http://www.vasculitis.org.uk/about-vasculitis/what-is-anca

The pathophysiology of the disease, or the way in which the disease works, revolves around something called ANCA. ANCA, ntineutrophil cytoplasmic antibodies (picture to the right), are antibodies made by your OWN body to attack your OWN cells and kick your immune system into overdrive against yourself, completely counterproductive and ridiculous really.







Neutophils - nucleus (powerhouse of the cell) in purple, cytoplasm light purple. https://www.verywellhealth.com/polymorphonuclear-leukocyte-2252099




The antibodies act on neutrophils - one of the white blood cells - which are part of the body's first line of defense against pathogens or "bugs". These antibodies are targeted against certain proteins in the cytoplasm of the neutrophil, ie tiny proteins found the jelly body of the neutrophil cells (see picture to the left).




Pathophysiology of C-ANCA vasculitis. https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anca-vasculitis/

There are 2 types of ANCA antibodies. P-ANCA antibodies are against proteins called myeloperoxidases (MPO). Normally the function is to produce oxygen radicals. Conditions linked to this antibody are MPA, EGPA and ulcerative colitis. The other antibody is C-ANCA, antiproteinase 3 positive (see first picture), meaning the antibodies attach to and trigger the proteinase 3 protein inside of the neutrophil cells. These are the antibodies that I have, mostly associated with Polyangiitis with granulomatosis (previously Wegner’s Granulomatosis). The result of these little buggers is triggering the neutrophil cells to target and attack blood vessels. The neutrophils recognise the cells of the blood vessels as non-self and begin to attack them, thus causing inflammation and necrosis (death) of the cells of the small and medium blood vessels leading to vasculitis and an endless list of symptoms and complications. (2)



What are the symptoms to look out for?

Unfortunately many of the symptoms are very non specific and therefore it takes countless time, tests, skilled doctors and a high index of suspicion to make the diagnosis. Constitutional symptoms (the bane of my life) include fevers, night sweats, weight-loss, coughing, general malaise and fatigue - so not very direct symptoms at all. Other symptoms include muscle pain and weakness, non-specific pain, coughing up blood, breathing difficulties, tingling sensations and numbness throughout the body, seizures, skin lesions, arthritis and eye involvement, to name a few.


*SIDE NOTE* I'll have a category called Autoimmunity in which I will share posts relating to this disease, how it's affected me, my management program, the general journey it's taken me on this far, autoimmunity in general and some more scientifically based stuff. To follow more of these posts subscribe to the blog.

But for now I'm sticking to the facts and stone cold evidence of this devastating disease.


ANCA associated Vasculitis is a relapsing and remitting disease, meaning it consists of cycling through periods of being well and feeling okay intertwined with having "flares" or bouts of illness, in which one can become totally incapacitated and exceptionally unwell. Medically we use similar terminology for autoimmune conditions as we do when we talk about cancer, they sometimes use similar medications to manage them too. We say the disease can go into remission, in this case the markers in the blood, such as C-ANCA or P-ANCA, ESR, CRP, IL-6 which are all markers of the disease and show inflammation within the body, have normalized, there is no evidence of disease seen and the patient feels well. However, the disease can easily reactivate and begin to wreak havoc once again - ie a relapse or a flare. A vicious cycle that continues throughout one's life. Multiple relapses and poor control of the disease are associated with extensive complications including; kidney damage and renal failure, irreversible damage to the lungs and respiratory failure, aneurysms and strokes etc. But it's not all doom and gloom, and with the advances in medicine combined with lifestyle changes and integrative practices, it IS possible to gain control of the disease and prolong remission.


Let's talk treatment.

First and foremost, it is important to note that this disease is dangerous and without adequate management, it is life threatening and completely devastating.


How do we stop the body from attacking and eventually killing itself? Well my cherished readers, we throw big, scary drugs at the disease in an attempt to suppress the immune system and stop it from attacking itself. The mainstay of treatment that rheumatologists will put you on includes; a combination of steroids, chemotherapeutic drugs (the same "chemo" they use in cancer treatment but at a lower dose, some adjunctive newer medications that are added to the protocol (Hydrochloroquine and Purback to name a few) and some other things to help protect the body from the side effects of these meds. A newer agent known as Ritixumab has made it's way into the protocol. This medication is also used in certain cancers - specifically lymphomas and leukaemias- however, it's not a chemotherapeutic agent, rather it's a monoclonal antibody which attaches to the CD20 proteins or receptors on cells within the blood and tags these cells to be destroyed by the immune system, as well as playing a roll in destroying them itself. These medications all act to suppress the immune system and decrease it's ability to attack itself. (2)


The side effects of these medications can be vast, depending on the dose and the response of the individual. With their ability to suppress the immune system, they leave you somewhat immuno-compromised and at risk of developing illnesses very easily, they can cause fatigue, nausea, headaches, dizziness, diarrhoea and vomiting, drop the white cell count, increasing the likelihood of secondary infections, as well as haemaglobin causing anaemia, etc. None of these lend themselves to making my work in the hospital any easier, quite the contrary actually.


While the conventional treatment is essential and has a vital role to play in helping patients with this disease, it didn't resonate with me and left me feeling exceptionally uneasy. I am unbelievably fortunate to know some pretty incredible doctors and I have many resources at the tip of my fingers, so I have chosen a more alternative treatment plan for myself, which doesn't involve any chemo agents and relies on supporting and nourishing the body back to health. I've basically made myself a guinea-pig to try and find another way to manage the disease, one that combines the two worlds I spoke about in my previous post, and supports my "path to becoming a healer." So far so good, let's hope it works! All in the name of science and all that jazz.


It all sounds pretty complicated I'm sure, and had it not been for my medical background, I'm convinced I would have been left dumbfounded by this diagnosis and all the weird and wonderful terminology that comes with it. I'm in a privileged position to be able to understand my disease at a scientific level, truly grasp what's happening, and seek out the correct information when there is an aspect that I may find difficult to comprehend. This is what drove me to write this blog, to try and help others who don't have the same privileges, to create a place where others who are going through similar experiences can seek guidance and answers and no longer feel alone and confused. Something I've learnt, which I believe is an important lesson is that a disease should never define you, but should be viewed as a major catalyst for personal growth and healing.


More information will be coming soon in my other posts, however, feel free to contact me if you have any questions or you need anything else.


Sending love,


Cayla


References:

1. Pagnoux, C. (2017). Updates in ANCA-associated vasculitis. [online] pubmed. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5058451/ [Accessed 2 Feb. 2019].

2.UNC school of Medicine. (2019). ANCA Vasculitis | UNC Kidney Center. [online] Available at: https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anca-vasculitis/ [Accessed 2 Feb. 2019].

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