RA or PR? Or both?

Polyanna Penguin’s post today snagged my attention. In it, she asks people who’ve been diagnosed with palindromic rheumatism to get in touch with Arthritis Research UK, an organization in Great Britain that’s researching the disease.

The term set a bell jingling in my mind, so I googled it.

Palindromic Rheumatism (PR) is a rare autoimmune disease. “The descriptor ‘palindromic’ is used based on its Greek root palindromos, which means ‘to come and to come again,’ which aptly describes this phenomenon,” states Dr. Gordon Lam in a paper he wrote about the disease for Johns Hopkins. “… palindromic rheumatism is an idiopathic, periodic arthritis characterized by multiple, transient, recurring episodes of mono [single joint]- or oligo-[up to four joints] arthritis associated with tissue swelling around the involved joints. Episodes last for a few hours or a few days and then spontaneously resolve. Between episodes, there are no residual effects.”

SwellyHand2Bingo. I read Dr. Lam’s paper with growing recognition—and astonishment. His description of the disease, along with the story of one of the patients he’d diagnosed with PR, sounded incredibly familiar.

I was diagnosed with RA in 1987 after nearly a year of transitory, often disabling joint pain that came and went without warning and without, as far as I could tell, reason. The flares affected one joint at a time and could occur on either side of my body. They moved around. One day, it would be one of the knuckles on my left hand that was hot, swollen and so painful that I couldn’t bear to move it. The next day it would be my right knee, or my left great toe, or my right shoulder, or my left jaw or… or… or. You catch my drift. The flares started suddenly, lasted anywhere from two hours to four days, and ended just as abruptly. I never knew from one day to the next what to expect. Usually there would be a break—sometimes of up to two weeks, and the flares could vary in intensity.

The only continuous pain I experienced was on the bottoms of my feet. From the moment I got out of bed in the morning, my feet would feel as if I’d been standing on them all night long. They were sore and tender, and the first steps I took usually elicited groans of pain. Throughout the day, walking hurt, but standing for any length of time hurt even more. Still, I functioned, forcing myself to endure the pain and get on with my day, my professional work, and my life as a wife and mom.

When one of my joints flared, it would be exquisitely, excruciatingly painful. I could not move it nor bear much if any weight on it. Sometimes even the lightest pressure could be agonizing. For instance, if the flare was in one of the joints in my foot, I would have to hang it off the edge of the bed, uncovered, in order to rest or sleep at night. Even a sheet was too much. I often had to use a cane and, more than once, was prescribed crutches by my doctor for a week or two at a time.

I was a civilian married to a soldier and worked for the U.S. Army in Germany at the time, so my medical care was provided by the military. I was very lucky. My doctor, a young internal medicine specialist, believed me regarding the strange, awful pain I was experiencing and tested my blood for the rheumatoid factor first thing. There it was. He diagnosed me with rheumatoid arthritis and began treating me with NSAIDs, starting with high-dose aspirin.

Over the next five years I had blood test after blood test. Sometimes the Rf factor showed up, sometimes it didn’t. I learned that a diagnosis of RA required the satisfaction of several criteria: that the Rf factor show up in the blood; that there was symmetrical involvement of several different joints; and that flares would last for three weeks or more.

None of them ever really applied to me, as far as I could tell. My flares never occurred in the same joints on both sides of my body at the same time (re: flares in both knees or both wrists or both ankles at the same time). My flares didn’t last for weeks, but for hours or days, usually with short, flare-free periods that lasted a day or a week in between.

I also didn’t have any of the other criteria: I wasn’t unduly fatigued, I suffered no weight loss (darn!) and I never ran a low-grade fever or experienced any flu-like symptoms.

The exception was my near-continuous foot pain, which was symmetrical and long-lasting. But my doctor had no explanation for that.

It has always niggled at me that my symptoms didn’t exactly match the RA diagnostic criteria. But my doctor was the only expert I knew. He said I had rheumatoid arthritis. I believed him.

During those years, none of the NSAIDs or DMARDs—gold salts and plaquenil—ever worked to relieve my pain. The only drugs that did were narcotic pain relievers, and those were doled out with great caution, just 10 pills at a time. I was very frustrated and in a great deal of pain, and I missed a fair amount of work.

When we left Germany in 1992 and resettled in Washington state, I went to a new Army doctor. After a quick examination—I have no idea if he even looked at my medical records—he told me that my prior doctor had been wrong. I didn’t have rheumatoid arthritis, I had palindromic arthritis.

I was floored. What does that mean, I asked. He said that it meant that I had a type of arthritis that occurred in many different joints at different times, sort of a catch-all type of arthritis, and after all, there were many, many different types and they were all hard to diagnose. Anyway, since there was basically nothing they could do to relieve it, I’d just have to live with it. He didn’t offer any treatment or follow-up appointments. He was hostile and dismissive. And I was embarrassed to ask for narcotic pain relief, figuring he’d just think I was drug-seeking. He probably already did.

I left his clinic stunned… and after a while, absolutely furious. I was convinced that this new doctor had no real idea what I had and that he’d basically blown me off. Since the many different RA drugs I’d tried hadn’t had any effect on my RA (if that was even what I had!), I decided then and there that I’d never bother with another doctor, ever again, about my pain. I’d just, as the good doctor said, learn to live with it.

And that’s what I did for the next six years. Naproxen, the NSAID, was released as the over-the-counter pain reliever Aleve. I took it now and then. And by some stroke of luck, as time passed I had fewer and fewer flares. The pain gradually lessened in intensity. And finally, in 1999, the flares stopped entirely. I had achieved that fabled state, “remission.”

It lasted until 2005, when I developed two large, long, firm, mostly painless lumps beneath the skin along the outside of my right wrist. I consulted my PCP, who had no idea what they could be and who quickly referred me to an orthopedic surgeon. After X-raying my wrist, he asked if I’d ever had arthritis. I told him about my old struggle with “RA.” “Ah!” he said. “That explains it.” He said the lump was thickened, excess synovial fluid that had leaked out of the wrist joints and slowly hardened in place. Even though my RA was quiet, he explained, it was still in my body doing damage. If left alone, the lump would probably continue to grow and, eventually, impair the free movement of my wrist and hand. The other option would be to have it surgically removed. And he urged me to see my PCP again for a referral to a rheumatologist.

I had him remove the lump. Today I have a five-inch-long, flattened M-shaped scar that runs from the base of my palm up the outside of my wrist along my forearm. It healed without incident in about six weeks.

I was still angry and frustrated with doctors, however. I didn’t ask for that referral. I wanted nothing to do with rheumatology. I wasn’t having any flares or pain. Why bother?

It was in the years following the surgery that my hands began to swell and ache. And as that happened more and more often, and the pain slowly intensified, I finally broke down and went to a rheumatologist for treatment. I’d heard that new RA drugs had been developed. Maybe there would be something that worked, a wonder drug that would be worth the risk of the dangerous side effects.

My VA rheumy comfirmed my original doctor’s diagnosis of rheumatoid arthritis. He’s never once mentioned any other kind of arthritis to me. He treats my disease with a cocktail of DMARDs and tests my blood for the usual RA markers, including the Rf factor, which still shows up whenever he includes it in the tests. I’m not in remission anymore, but my disease activity is low.

As I’ve written here before, my old rheuma-dragon changed while we were in the Land of Remission. Instead of viciously sinking his long fangs into one joint at a time, he just chews and gnaws like a monster puppy at my knuckles and wrists—both hands at the same time. Once in a while he’ll give me a sharp nip in a knee or ankle, or he’ll sink his teeth into a shoulder for a while. The pain has only rarely been as intense as it was in the early years. Instead it’s mild to moderate and—a major difference—it’s constant. My hands always hurt. There is frequently some swelling around my knuckles, too. It’s like I’ve had a single, low-grade flare going on since about 2007.

I still don’t have fevers or mysteriously lose weight (darn it, again! If I have to have this disease, at least there might be one good thing about it!). I still don’t experience any flu-like symptoms. I do, however, become fatigued much more easily than I used to. It could be RA, but it could also be due to age—I’m 26 years older than I was when I was first diagnosed. And it could be from the hip bursitis my old rheuma-dragon brought with him when he woke from his long remission sleep. Some of the bursitis pain is a feeling of fatigued weakness in my hips.

So. What was my point about palindromic rheumatism?

As I read Dr. Lam’s paper on it this morning, it was like bells and whistles started going off in my head. I now believe that I probably did have palindromic rheumatism during those first, really bad five years. The Army doc I saw in Washington—the one that blew me off—was right, after all. I really wish he’d taken the time to explain to me how he came to that diagnosis, and I wish he’d had the patience, kindness and empathy to at least try to treat it, instead of telling me I just had to live with it. If he had, maybe I’d have gotten started on more effective treatments for it and for RA back in the early 90s.

Dr. Lam’s paper states that PR frequently precedes RA. It often goes into remission for a period of time and then reappears as full-blown RA. The Rf factor often shows up in blood tests in PR.

Finally, one of the puzzles about my RA is that I’ve had only very minor joint erosion over the years, mainly in my thumb knuckles. So many people with RA end up with terribly deformed, disabled joints.

It turns out that palindromic rheumatism never causes joint erosion. How about that!

I have an appointment with my VA rheumatologist later this month. I’m going to ask him about all this and see what he has to say. If I’m right, and I did have PR either alone or with my RA, questions I’ve had about my RA for years and years will finally be answered.

 

10 responses to “RA or PR? Or both?

  1. Wren, that is an interesting story and just adds to the idea all of us know that this RA dragon is a sneaky one and doesn’t always follow a predictable pattern. Please keep us posted on what you find out from the upcoming appt.

  2. Wow, what a crazy calamity of events…I will be keeping an eye on my own symptoms, although, my joint pain is always symmetrical and, just like you, hand pain every day. I will look forward to that dream of “remission”. I hope you will find your answers to help win the battle of your disease.
    J.G.

  3. I’m sorry you had such a hideous experience when you got back to the US! It’s fascinating though, isn’t it – I can’t help wondering if actually that’s what I’ve got (at the moment) which may mean I’ve got ‘proper’ RA to look forward to, which isn’t a cheery thought, but at the same time it would explain why I can still walk on my left knee after a series of really nasty flares! I’ll be very interested to hear what you find out at your next appointment – do keep us posted!

  4. Wren this was fascinating! I had never heard of this but I am really intrigued…I did not have a positive RA factor for over 5 years after being diagnosed and to this day (17 years later) I have no erosions! so interesting….thanks for sharing…your posts are always great!

  5. carlascorner

    An amazing twist to your journey. Knowledge about our condition(s) is valuable, even in hindsight. I had never heard of PR, but like you, over the course of the years I’d have a joint swell beyond use — then go completely back to normal until weeks/months later when a different joint would be affected. Was this PR — a precursor to my RA? Maybe. If course, being one of those sero-negative patients, sometimes I wonder (wish for?) if RA is the correct diagnosis. Sneaky, sneaky dragon! Thanks to you and PollyAnna Penguin for raising this interesting topic.

  6. Quite interesting story, I am sorry on what you had experienced, but I am pretty sure you overcome it. It is really better for us to know what are the basic symptoms of certain disease or health issue that’s why we can deal with it accordingly.

  7. Geez. I didn’t want to read that anymore. I went through a Stem Cell Therapy for my RA pains in the knees and the pain actually reduced. I couldn’t believe it my self but it worked for me and I hope it will work for you as well.

  8. Pingback: Puzzled but pleased … | RheumaBlog

  9. This entry leaves me teary-eyed. I’ve suffered mild and “random” asymmetrical joint pain for such a long time, and we found high rheumatoid factor years ago, but with no diagnosis I got tired of being treated like a hypochondriac. I am finally experiencing pain in the shoulders, elbows, and hips that seem to be what’s required for an RA diagnosis, so I finally made another appointment (for next month) with the rheumatologist. But I don’t ever have joint swelling, and my joint pain is most often asymmetrical. I also have fatigue that comes/goes (and edema and brain fog that seems to be helped by a vegan diet I find hard to sustain). I’m grateful for your blog entry (1) because it supports my quest to not give up even though I come across bad doctors or “good” doctors with less-than-ideal bedside manners, (2) lets me know about PA, something I’d never heard of before. Sorry, I don’t normally clog up someone’s blog comments region with my own story, but this is my eye-opening and possibly life-changing moment so please indulge me. I will ask about PA next month.

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