Hi everyone. For the last 6+ months I have been researching Wegeners like a fiend. I came across a few older posts, and rather than respond to those, I figured I would start a new post and share new information that my Drs have recently learned though me and another patient.
I was not diagnosed with Sjogrens until I was 39. About the same time my older sister was diagnosed with Lupus at 46. A few later in April 2015, my mother passed from Idiopathic Pulmonary Fibrosis. I would air quote that (if quotes worked on here) because she was never properly assessed for Sjogrens/Lupus, and no biopsy was taken from the lungs.
Flash forward to Memorial Day 2016, and after 4 fun filled days of Virginia wineries, I notice a hardened bump on my lower lip, almost at the same place the NIH took my lip biopsy a few years before. I figured it would heal. It didn?t. I tried to use a safety pin to coerce it to go...nothing. So.. I finally see my dentist in September; he says, oh it is probably just a ruptured salivary gland. I tell him I did not think I hit my mouth that hard with a wine glass... but you never know.
All kidding aside, I see the oral surgeon late October, he cuts it out... but assured me it?s nothing..just Sjogrens. Surprise! Pyogenic granuloma (which is a small version of a hemangioma) AND it reformed within 48 hours of extraction. I found the name of Wegeners by searching the (PG) and (Pulmonary Fibrosis). Problem was... I could not remember the stupid name of it when I was at my Rheumys office in the winter.
Moving onto March 2018 and St Patricks Day...I am dragging. I get up on Sunday the 18th, my throat is sore... I smell sinus infection (there is no other smell like it). By the 20th, everything is swelled shut in my ears, sinuses, throat; but I have no bronchitis. This is odd and the first time ever I have had no cough. For two weeks I blow nothing but blood. By day 10 of the 2 weeks... I am seeing a spot in my left eye (like I stared at a lightbulb for too long, but it will not go away). Then, the next day... it is 2 spots. I call the eye dr... fast forward 7 hours and an emergency retinal appt later; I find out I have multiple ruptures in my left eye (Retinal Vein Occlusion AKA a stroke!). I have been on low dose beta blocker and BP meds, and it has been healing great... but here is the best part of the story (and hopefully helpful for other people that have wondered about Wegeners and their Dr treats them like they are crazy).
I go back to the Rheumy, and ask for the ANCA bloodwork. She pushes back a little.. I remind her my mom died from IPF and I am 5 times more likely to do so (Wegeners causes IPF). She agrees Wegeners can be masked by Sjogrens and reminds me if its positive.. theres a tissue biopsy involved... I remind her I hang out in OR rooms for fun, she writes the bloodwork and a sinus CT order. I do the sinus CT along with my chest and abdominal CTs, cause I have cysts, nodules and benign masses growing on vital organs like it is just a part of life.
I go back back to see her with the CDs of my three CTs and knowing my ANCA is negative (gotta love Quests IPhone app) with a side of severe vertigo (oh yeah... I have major vestibular issues too), expecting her to (I told you so).
Instead she tells me she just had a patient with negative ANCA, but kept running fevers (story of my life). His spleen was removed recently and the biopsy came back positive for Wegeners. Knowing she had multiple patients trying to rule out/diagnose Wegeners, she called Johns Hopkins (my dr is part of the Fairfax INOVA system outside of Washington DC, so it?s realistic) and asked how it?s possible.
Hopkins said: unless there is renal involvement, ANCA is almost always negative. Tissue sample is required to confirm positive and negative.
She points to my Sinus CT report, and needless to say... it is a hot mess, especially right under the eye with the rupture. She is referred me for a sinus biopsy to rule out Wegeners. I have been waiting now for my appt with the ENT surgeon (July 19th).
Since then...I have gone back to the Retinal Specialist for a checkin. He used to teach at Hopkins. He said this ANCA result dependancy on renal involvement was news to him, and should my ENT surgeon refuse to biopsy, he (the Retinal Specialist) will get me into Hopkins, since they are publishing this guidance.
So, please.... do not give up and do not stop challenging your Drs. If you have a halfway decent one.. they will admit when they learn something new and will not hold your quest for a better quality of life against you.
Welcome to the forum
Amen and Amen girl!!!! I did a whole lot of research on Wegeners years ago because I had so many sinus infections and weird sinus drainage....lots of really weird stuff along with aching and pains all over and fatigue big time. I argued with doctors and they would not believe me when I told them there was really something wrong with me ad probably autoimmune. I never thought of Sjogrens as even as a registered nurse my training taught me dry eyes and dry mouth.
The long story is I did not have Wegeners but after many years of doctoring (like 40) I was diagnosed with Sjogrens in 2003 (had been diagnosed with Bullous penmphigoid in 2002 but no doctor paid anyt attention to that. Remember-----one autoimmune disease can have another!!!! Well, still felt lousy and had a mycoplasma infection and ob 8 antibiotics for one year. Finally referred to immunologist and found to have Myasthenia gravis, Hashimotos and severely low t-cells and IgG levels. Sooo, yeah, I was sick with autoimmune and the Mayo clinic told me I wasn't sick and did not have Sjogrens plus they ignored my positive anti striated muscle antibody which indicated myasthenia. Twp years ago I added autoimmune ear disease which is taking my hearing. I had lost one ear 20 years ago and now have about 50% in the other and it is slowly getting less.
Now, You are so right about persevering in looking for diagnosis. I argued and was insulted and ignored for so many years. I changed doctors so many times and just would get so down. I had more infections from tooth extractions (lost all my teeth eventually) and MRSA and a whole lot more. I am still surprised that I am still alive. Last night I counted up and I have 11 specialists who I am well acquainted with. My main ones are all in one clinic and are very good to me and work with me. They still shake their head though. They also listen because they tell me I am really tuned into my body and the little things it does when it gets worse.
So, people, do not give up. Continue to push for diagnosis and take someone with you to appointments as much as you can. We all need validation and support----especially when we are still seeking diagnosis. My hubby came with me and had to speak up very frequently in my behalf. My hubby did go through a stage where he was sick and tired of taking me to the doctor cause they never did anything. My response to him was I will go alone because I want to know what they are going to put on the death certificate.
If I remember correctly Wegeners has had a name change which I can't remember and it affects the larger blood vessels causing vasculitis of the organs. The Sjogrens affects the waller vessels but can still be serious. Thanks so much for your info. Irish