In depth
Relapsing polychondritis: common questions
Somebody searched the whole published literature for a guideline in this disease and turned up 20 citations. Not one of those 20 citations was a guideline, so what the group produced instead was a list of eleven things the field still needs. That list is the most useful document in relapsing polychondritis, and this page starts from it.
What the research found.
A European reference network searched for guidelines in relapsing polychondritis and the search turned up 20 citations. Not one of those 20 was a clinical practice guideline, which is a striking result for a disease described in the literature for decades. So eleven areas of unmet need were listed instead of recommendations.
One guideline covers every adult going onto a long course of corticosteroids, which is the 2017 American College of Rheumatology guideline. It strongly advises getting enough calcium and vitamin D, and it asks for weight-bearing and strength work alongside them rather than leaving the kind of exercise open. It also advises stopping smoking and limiting alcohol, at any age and whatever put the person on the drug.
One study followed 295 patients with this disease and found airway trouble in 70.5 percent of them. The voice box was the most common spot at 82.2 percent, and 25.7 percent of the patients whose voice box was involved needed a hole made in the windpipe. The main cause of death in that cohort was the airway closing.
One hospital looked at its own inpatients with this illness and found the heart involved in 24.1 percent of them. Its authors call regular heart tracings and heart scans necessary in this disease. That's one hospital and those were admitted patients, so the figure deserves holding loosely even while the recommendation is still in place.
No guideline exists, and somebody checked
A European reference network went looking for clinical practice guidelines in relapsing polychondritis, and it searched the literature properly rather than glancing at it. The search turned up 20 citations in total, and not one of them was a guideline. For a disease that has been described in the literature for decades, that's a startling thing to find.
What came out of that work instead was a list of eleven areas the field needs, which is a different kind of document altogether. It covered how to diagnose the disease and how to treat it. It covered pregnancy, childhood-onset disease, how often to follow up and screen, surgery and anesthesia because this disease reaches the windpipe, cancer risk, and preventing infection.
Read that list as the field's own account of what it doesn't know. The only alternative on offer anywhere is borrowed advice dressed up as settled, and that's a worse thing to hand a newly diagnosed reader. It's why this page reads the way it does, and why there are so few numbers on it.
Why the airway keeps coming up
Surgery and anesthesia got their own entry on that list for a straightforward reason. This disease inflames cartilage, your windpipe is cartilage, and that makes airway involvement a known and serious feature rather than an odd complication. It's the single thing that sets this illness apart from most of the rheumatic diseases on this site.
Two things follow from that, and the first is that anesthesia and surgery here need planning rather than routine handling. So raise it with any surgeon or anesthetist yourself, because it may not appear in your notes at all. The second is that breathlessness, a change in your voice, or noisy breathing all need seeing promptly, and not one of them belongs under being out of condition.
Nothing else on this page counts as much as that pair does. The lifestyle research in relapsing polychondritis is too thin to quote from, and in a disease where that's true the airway is where your attention belongs. Everything below is worth reading, and nothing in it displaces the two paragraphs above.
Why there's no exercise figure here
The published exercise work in this disease covers a few people at a time, and not one of those compared a program against anything. So not one of those can tell you whether training changes what you're able to do. That's a real limitation of the studies rather than a judgment about the people who conducted them.
We've left those numbers off the page, and printing them with a warning attached would not have been better. A figure drawn from seven people appears on a page looking just like a figure drawn from seven thousand, and a reader has no way to tell the two apart. So it's better to know the research isn't there yet than to be handed a number that can't hold the weight put on it.
That's no reason to sit still, and the corticosteroid guideline below still applies to most people here. It asks for weight-bearing and strength work, which is more specific than anything the disease literature offers. What this illness adds on top of the guideline is the airway, and that's a reason to watch your breathing rather than to stop moving.
What applies through the treatment
Most people with this diagnosis take corticosteroids, and that brings the one firm recommendation available here. The 2017 American College of Rheumatology guideline covers every adult going onto a long course of them, at any age. It strongly advises getting enough calcium and vitamin D, doing weight-bearing and strength work, stopping smoking, and limiting alcohol, and those four items are its own wording.
That reaches you through the drug rather than through the disease, and it applies whatever put you on a long course. Two words in it are worth keeping hold of, which are weight-bearing and strength. They name a kind of work rather than leaving you to guess at one, and guessing is what most advice in rare disease asks of you.
One study of the newer drugs watched patients instead of testing anything, across 41 people and 105 courses of biologic treatment. In the first 6 months 62.9 percent responded in some way and 19.0 percent responded completely, and then 73.3 percent of those courses were stopped, a third of them for the drug not working well enough. There was no comparison group, so not one of those figures separates the drug from the illness quieting down on its own.
No trial of any diet exists in this disease, so consider what a diet sold for relapsing polychondritis is competing with. It's competing with a literature of 20 citations and no guideline at all. That's a low bar rather than a recommendation, and clearing a low bar tells you very little about whether something works.
Living without a map
With no guideline, more rests on your individual clinician than it would in a commoner disease. More rests on what you report too, so there's a case for staying with one team rather than moving around, and a case for asking what any recommendation is based on. There's also a case for describing changes in detail rather than in general terms, because the detail is what a clinician has to work from.
The symptoms worth mentioning follow the disease, which targets cartilage wherever it happens to be. So report ear or nose pain and swelling, changes in hearing or balance, red or painful eyes, and joint pain. Above all, report any change in breathing or in your voice, for the reasons set out near the top of this page rather than for anything on the rest of it.
This isn't satisfying if you came here looking for a plan, and it isn't meant to be. What this page offers instead is an honest account of what's known, which is thinner than anybody wants it to be. The specialists who looked hardest concluded that the field needs eleven things it doesn't have, and knowing that is better than being handed confident advice with nothing behind it.
Common misconceptions.
Myth. There must be a guideline for this somewhere.
Reality. Somebody searched the whole literature and turned up 20 citations, not one of them a guideline. What came out of that work was a list of eleven things the field needs, covering how to diagnose the disease and how to treat it. They also cover pregnancy, disease starting in childhood, how often to follow up and screen, surgery and anesthesia, cancer risk, and the prevention of infection.
Myth. Exercise research exists for this condition.
Reality. Not any this site will quote, because what's published covers a few people at a time. Not one of those studies tested a program against anything, and work that small can't tell the disease apart from being out of condition or from the corticosteroids. So we give you no figure from it, since a weak number reads as firm once it's printed on a page and that's where the harm is.
Myth. Reduced exercise capacity here is just being unfit.
Reality. It may be, and it may not be, which is why it's worth assessing. This disease inflames cartilage and that includes your windpipe, so windpipe trouble here is serious enough that the unmet-needs list gives surgery and anesthesia their own entry. So breathlessness here deserves an assessment rather than a training plan.
Myth. Advice for other rheumatic diseases applies to me.
Reality. It's what gets used, because there's nothing else, and it also hasn't been tested here. This disease inflames cartilage in the ears, the nose, the joints, and the airway, which is a different spread from any disease whose guidelines get borrowed. Note what the reference network did about the absence, because it listed what needs studying and recommended no borrowing.
Questions patients ask.
Are there guidelines for relapsing polychondritis?
No, and a search turned up 20 citations in the whole literature and not one of them is a clinical practice guideline. In their place, eleven areas of unmet need were listed. Those cover how to diagnose it and how to treat it, pregnancy and disease starting in childhood, how often to follow up and screen, surgery and anesthesia because this disease reaches the windpipe, cancer risk, and preventing infection.
Why does surgery get its own entry on that list?
Because this disease inflames cartilage and your windpipe is cartilage. One study of 295 patients found airway trouble in 70.5 percent of them, which makes anesthesia and surgery a specific problem here rather than a general one. It's why a group listing unmet needs picked it out among eleven items. So tell any anesthetist or surgeon that you have this diagnosis, because it may not be in your notes at all.
How likely is my airway to be involved?
More likely than most people expect, on the one study that followed 295 patients. Airway trouble appeared in 70.5 percent of them, and the voice box was the most common spot at 82.2 percent. A quarter of the people whose voice box was involved needed a hole made in the windpipe, which is 25.7 percent of that group. Two things raised the risk, which were being younger when it started and having breathing symptoms first.
Should my heart be checked?
There's a case for it, from one hospital that looked at its own inpatients with this illness. The heart was involved in 24.1 percent of them, and its authors call regular heart tracings and heart scans necessary here. That's one hospital and those were admitted patients, so the figure is worth holding loosely. It's still a fair thing to raise at a visit with your team.
What's known about exercise capacity here?
Less than you'd want, because there's no trial and the published work covers a few people at a time. It wasn't built to test anything, so this page gives you no figure for it. What applies to you reaches you through your treatment instead, and that part of the answer does have a guideline behind it.
Has anyone tested an exercise program?
No, and nothing published in this disease compares a program against anything. Reports on one or two people can't tell a training effect from the disease settling down on its own, which is what makes them unusable for this purpose. So there's no before and after figure to give you, and inventing a plausible one is the thing this site won't do.
Should I be exercising?
The reasons everybody has still apply, and one firm recommendation covers most people with this diagnosis on top of them. Anyone on a long corticosteroid course falls under the 2017 American College of Rheumatology guideline, which strongly advises weight-bearing and strength work. It advises calcium and vitamin D alongside that, plus stopping smoking and limiting alcohol. All of that reaches you through your treatment rather than through your disease.
Is there a diet for this condition?
No trial of any diet exists here, and what applies instead is the corticosteroid guideline above. So think about what a diet sold for relapsing polychondritis is competing with, which is a literature of 20 citations and no guideline at all. That's a low bar to clear rather than a recommendation of any kind. Clearing it tells you nothing at all about whether the diet does anything for you.
Why is there so little research?
The disease is rare, so gathering enough people for a trial is genuinely hard, and the research effort that exists goes to diagnosis and treatment. The network exercise that produced the unmet-needs list exists because somebody recognized the absence. What it produced was a list of what needs doing rather than a set of answers.
What counts most in managing this?
Getting your airway watched, which is what the specialists flagged when they wrote their list. Windpipe involvement is why surgery and anesthesia appear on the unmet-needs list, and it's the feature that sets this disease apart from most rheumatic disease. So new breathlessness counts, a change in your voice counts, and noisy breathing counts. Those need seeing promptly rather than waiting for the next routine appointment.
What should I take to my next appointment?
Any change in breathing or voice comes first, for the reasons above. Beyond that, several things count, and they are ear or nose pain and swelling, changes in hearing or balance, red or painful eyes, and joint pain. This disease targets cartilage wherever it is, and there's no guideline here to fall back on. So what you report does more work here than it would anywhere else on this site.
References.
- Rednic S; Damian L; Talarico R et al. Relapsing polychondritis: state of the art on clinical practice guidelines. RMD Open. 2018;4:e000788. 10.1136/rmdopen-2018-000788Systematic literature review for clinical practice guidelines in relapsing polychondritis
- Buckley L; Guyatt G; Fink H et al. 2017 American College of Rheumatology Guideline for the Prevention and Treatment of Glucocorticoid‐Induced Osteoporosis. Arthritis & Rheumatology. 2017;69:1521-1537. 10.1002/art.40137GRADE-based clinical practice guideline
- Chen N; Zheng Y. Characteristics and Clinical Outcomes of 295 Patients With Relapsing Polychondritis. J Rheumatol. 2021;48:1876-1882. 10.3899/jrheum.210062Retrospective cohort of 295 patients with relapsing polychondritis at Beijing Tongren Hospital
- Yin R; Zhao M; Xu D et al. Relapsing polychondritis: focus on cardiac involvement. Front Immunol. 2023;14:1218475. 10.3389/fimmu.2023.1218475Screening of patients with relapsing polychondritis hospitalised between December 2005 and December 2021 at Peking Union Medical College Hospital
This page gathers the published research on this subject into one place. The studies behind it were published between 1989 and 2026, and every figure links to the paper it came from. Those studies were peer reviewed. This summary of them was not. Dr. Sarah Luebker is reviewing these pages one at a time and has not reached this one yet, so it carries no medical review date and nothing here is her opinion or her advice to you. Each page gets updated as she reaches it. It is here in the meantime because the science is worth having in one organized place that is easy to find and easy to read. Talk to your own clinician before acting on any of it.