Polychondritis—usually called relapsing polychondritis (RP)—is a rare disease where your immune system mistakenly attacks your own cartilage. Cartilage is the strong, flexible tissue that gives shape and support to parts of your body like the outer ears, nose, voice box (larynx), windpipe (trachea), ribs, and joints. In RP, this attack happens in flare-ups (relapses). During a flare, the cartilage gets inflamed, becomes red, hot, swollen, and painful, and, if the inflammation returns again and again, the cartilage can weaken or collapse. The disease can also inflame proteoglycan-rich tissues (things with a lot of cartilage-like molecules), such as the eyes, inner ears, heart valves, and blood vessels. RP is immune-mediated (driven by the immune system) and often behaves like an autoimmune disease. There is no single blood test that confirms RP; doctors diagnose it from the pattern of signs and symptoms, supported by tests and sometimes a small biopsy of affected cartilage. MDPIPMCCleveland Clinic
Polychondritis is a rare immune-system disease where the body mistakenly attacks its own cartilage and a few tissues that are rich in similar molecules (like the eyes, inner ear, and large blood vessels). Cartilage is the flexible tissue that shapes the ears, nose, voice box and windpipe, and cushions joints. In polychondritis, inflammation comes in bouts (relapses) separated by quieter phases (remissions). Over time, repeated swelling can weaken or deform cartilage, especially in the ears, nose, and airway. Doctors call the most typical form relapsing polychondritis (RP). There are no universally accepted treatment guidelines yet, and most therapies are based on case series, registries, and expert practice rather than large randomized trials. BioMed CentralACR JournalsPMC
A classic set of clinical rules called the McAdam criteria (later refined by Damiani & Levine and Michet) helps doctors recognize RP. These criteria include features such as auricular (ear) chondritis sparing the soft earlobe, nasal chondritis, laryngotracheal chondritis, eye inflammation, non-erosive seronegative arthritis, and audio-vestibular damage; meeting combinations of these features supports the diagnosis. BioMed Central
Why it happens
In RP, the immune system loses tolerance to parts of cartilage. Studies have found antibodies to type II collagen (the main collagen in cartilage) and to other cartilage proteins in some patients, and certain HLA genes (like HLA-DR4) raise risk, suggesting a genetic immune tendency. Still, the exact cause is unknown, and no single antibody or gene explains every case. PubMed+1PMCMedscape
A newer discovery is that a small but important subset of people with RP actually have a condition called VEXAS syndrome (a bone-marrow disorder caused by a UBA1 mutation, typically in older men). These patients may have macrocytic anemia, low platelets, and RP-like cartilage inflammation. Recognizing VEXAS matters because management and risks are different. PMC
Types
Doctors describe RP in a few practical “types” or patterns. These patterns help anticipate which organs might be involved.
By association
Primary (isolated) RP: RP occurs on its own, with no other autoimmune disease. BioMed Central
Overlap/secondary RP: RP appears with other autoimmune or inflammatory diseases (for example, systemic vasculitis, rheumatoid arthritis, lupus, Behçet disease, inflammatory bowel disease) or with some blood disorders (especially myelodysplastic syndrome). PMC+1
By phenotype (organ-dominant patterns)
Airway-predominant type: The larynx, trachea, and bronchi are the main targets. Breathing symptoms can be serious because airway cartilage can soften (tracheobronchomalacia) or narrow. Wiley Online Library
Hematologic-associated/systemic type: RP with blood or bone-marrow abnormalities (including VEXAS or MDS). PMC+1
Mild/systemic inflammatory type: RP with ear/nose/joint/eye involvement but without major airway or marrow disease.
Research groups have described three clinical phenotypes and, in other cohorts, four patterns with “limited” vs “systemic” forms. These are descriptive tools rather than rigid categories. Journal of Thoracic DiseaseBioMed Central
Causes
Important note: RP’s exact cause is unknown. The items below are factors, mechanisms, or disease associations found in research that likely contribute to RP. Not every patient will have these.
Autoimmune mis-recognition of cartilage – the immune system attacks cartilage components, causing inflammation and damage. PMC
Antibodies to type II collagen (CII) – present in a subset of patients, often higher during flares, supporting an immune role. They are not a perfect diagnostic test. PubMedMDPIACR Meeting Abstracts
Antibodies to other cartilage proteins – such as collagen IX/XI, matrilin-1, and COMP; these further suggest cartilage-targeted autoimmunity. SpringerLinkMDPI
Immune-complex deposition and complement activation – immune complexes and complement may participate in cartilage injury. PubMed
T-cell–mediated inflammation – cellular immunity (T cells) is thought to drive ongoing inflammation with lymphocyte-rich infiltrates on pathology. Medscape
HLA-DR4 genetic susceptibility – carrying HLA-DR4 (and some related class II alleles) increases risk, but does not guarantee disease. PubMedMedscape
Other HLA class II signals – studies in different populations find additional HLA associations (e.g., DQB1 variants), suggesting complex genetics. PubMed
VEXAS syndrome (UBA1 mutation) – clonal marrow disease that can present with RP features in older adults, especially men, often with macrocytic anemia. PMC
Association with myelodysplastic syndrome (MDS) – RP can coexist with MDS more often than expected by chance. PMCScienceDirect
Association with systemic vasculitis – overlap with vasculitic diseases (e.g., GPA) occurs in a significant minority. Wikipedia
Association with rheumatoid arthritis (RA) – some patients have both RP and RA, likely reflecting shared immune pathways. Wikipedia
Association with lupus and connective-tissue diseases – SLE, mixed connective-tissue disease, scleroderma, and Sjögren’s may accompany RP. PMC
Association with Behçet disease – inflammation patterns overlap in some patients. PMC
Association with inflammatory bowel disease – ulcerative colitis and Crohn’s disease have been reported with RP. ClinMed Journals
Association with psoriasis/dermatologic autoimmunity – skin autoimmunity sometimes co-travels with RP. Wikipedia
Possibly infection-triggered flares (indirect) – infections may non-specifically “wake up” the immune system and provoke a relapse (evidence is suggestive, not definitive). PMC
Mechanical stress/trauma to cartilage (indirect) – minor trauma may expose cartilage antigens and help trigger immune recognition in predisposed people (theory). BioMed Central
Age-related immune changes – many patients are diagnosed in mid-to-late adulthood, implying immune aging might play a role. PMC
Environmental factors (unknown specifics) – research suspects environmental cofactors but has not identified a single clear agent. PMC
Rare malignancy associations – overall cancer association is uncertain, but hematologic links (especially MDS) are the most credible. ClinMed Journals
Symptoms
Symptoms vary a lot from person to person and from flare to flare. Many have ear or nose symptoms first; others have joints, eyes, airway, or hearing problems early on.
Painful, red outer ear that is warm and tender; the soft earlobe is usually spared, which is a classic clue for RP. PMCMerck Manuals
Ear swelling that may come and go; over time, repeated attacks can change ear shape. Cleveland Clinic
Nasal pain and tenderness; repeated flares can weaken nasal cartilage and cause a saddle-nose appearance. Cleveland Clinic
Hoarse voice or voice changes if the laryngeal cartilage is inflamed. Cleveland Clinic
Cough, wheeze, or noisy breathing (stridor) when the windpipe or bronchi are inflamed or softened. Wiley Online Library
Shortness of breath, especially with activity, if the airway narrows or collapses. Wiley Online Library
Chest-wall pain from inflamed rib cartilage (costochondritis). Cleveland Clinic
Joint pain or swelling (usually non-erosive, seronegative arthritis) that migrates or flares. BioMed Central
Eye redness and pain from episcleritis or scleritis, sometimes uveitis; severe scleritis can threaten vision if untreated. PMC
Light sensitivity or blurry vision during eye flares. PMC
Hearing loss, ringing (tinnitus), or vertigo from inner-ear involvement. e-rvs.org
Skin rashes or tender nodules in some people (varied patterns reported). PMC
Fatigue, fever, weight loss during active inflammation. Wikipedia
Heart or vessel problems (less common): aortic root inflammation or leaky valves can cause chest symptoms or breathlessness. Cleveland Clinic
Low blood counts (anemia, low platelets) when RP coexists with marrow disorders like VEXAS or MDS. PMC+1
Diagnostic tests
There is no single “RP test.” Doctors combine your history, physical exam, targeted manual maneuvers, laboratory/pathology studies, electro-physiologic tests, and imaging to support or exclude RP and to look for complications or associated conditions.
A) Physical exam
Ear inspection and palpation – looks for red, tender auricular cartilage with earlobe sparing (a helpful RP clue). PMC
Nose inspection – checks for tender nasal bridge, swelling, or collapse. Cleveland Clinic
Airway/voice assessment – listens for hoarseness, stridor, or wheeze that suggest laryngeal or tracheal chondritis. Wiley Online Library
Ocular surface exam – checks for episcleritis/scleritis (red, painful eye) or uveitis signs. PMC
B) Manual tests (done gently by clinicians)
Pinna compression (“tender cartilage with earlobe sparing”) – gentle pressure over the cartilage hurts, but the soft lobule is usually unaffected. PMC
Costochondral point palpation – gentle pressure over rib–cartilage junctions reproduces chest-wall pain in costochondritis. Cleveland Clinic
Bedside hearing checks (Weber/Rinne with tuning fork) – screens for sensorineural vs. conductive loss during ear involvement; formal audiology follows if abnormal. PMC
C) Laboratory & pathological tests
Inflammatory markers (ESR, CRP) – often elevated during flares but can be normal. Wikipedia
Autoimmune screen (ANA, RF, others) – helps find overlap diseases (e.g., lupus, RA, vasculitis) that travel with RP. PMC
Cartilage autoantibodies (e.g., anti-type II collagen) – may be present in a subset; supportive but not diagnostic. MDPIACR Meeting Abstracts
Complete blood count (CBC) – looks for anemia, macrocytosis, or low platelets, which can suggest VEXAS/MDS links. PMC
Cartilage biopsy (ear or nose, only if needed) – shows chondrolysis and perichondritis with mixed inflammatory cells; used thoughtfully due to cosmetic/risks. Medscape
Basic metabolic tests/urinalysis – screens for organ involvement or treatment safety; kidney issues are uncommon but possible. PMC
D) Electrodiagnostic / physiologic tests
Pulmonary function tests (PFTs) with flow–volume loops – can show variable extra- or intrathoracic airway obstruction typical of laryngotracheal disease; helpful to monitor severity. MedscapeMayo Clinic Proceedings
Formal audiology (pure-tone audiogram/tympanometry) – documents hearing loss patterns during ear flares. PMC
E) Imaging & endoscopy
CT scan of the neck/chest (inspiratory–expiratory or dynamic) – evaluates tracheal/bronchial wall thickening, calcification, stenosis, or collapse; a key test in airway disease. PubMed
Flexible laryngoscopy/bronchoscopy – directly visualizes inflamed mucosa, erosions, ulcers, and stenosis; reserved for experienced teams. Journal of Thoracic Disease
Echocardiography – checks for aortic root dilation or valve regurgitation when heart involvement is suspected. Medscape
MRI (targeted) – shows soft-tissue inflammation (e.g., airway, ear, nose) and complications without radiation exposure. BioMed Central
FDG-PET/CT – can highlight active inflammatory sites (e.g., airway, large vessels) and help map disease extent when routine imaging is unclear. Medscape
Non-pharmacological treatments
Each item includes what it is, its purpose, and how it helps.
Education & flare plan: Learn your triggers, early signs, and who to call; having a plan lowers emergency risk and stress.
Cartilage protection (ear/nose guards, soft headsets, avoid piercings): reduces mechanical irritation that can spark local flares.
Smoking cessation: essential for airway-predominant disease; smoke irritates and weakens airway tissues.
Airway humidification (home humidifier, saline sprays): soothes inflamed mucosa, thins secretions, and eases cough.
Voice care (voice rest during flares, hydration, speech therapy): reduces trauma to an inflamed larynx.
Pulmonary rehab / breathing training: teaches diaphragmatic breathing, pacing, and airway clearance to reduce breathlessness.
CPAP/BiPAP (non-invasive positive pressure) when prescribed: pneumatic “splinting” can support collapsible airways and improve sleep and daytime function.
Activity pacing with graded exercise: maintains fitness without provoking breathlessness; joint-friendly movement (walking, cycling, water therapy).
Heat/ice for focal pain: gentle warmth/ice reduces ear, nose, or joint tenderness without systemic side effects.
Eye protection and lubrication (sunglasses, artificial tears): shields inflamed eyes from light and dryness.
Nasal saline rinses: gentle irrigation reduces crusting and discomfort.
Infection prevention (hand hygiene; prompt care for chest infections): airway infections can tip a fragile airway into a flare.
Vaccinations (up-to-date, non-live vaccines preferred once immunosuppression is planned): reduces infections that trigger flares; sequence with your clinician.
Stress reduction (CBT, mindfulness, supportive counseling): stress can amplify symptoms and coping burden.
Sleep optimization: adequate sleep supports immune balance and daytime energy.
Bone protection habits (weight-bearing exercise, calcium/vitamin D as advised): buffers the bone-thinning effects of long-term steroids. Office of Dietary Supplements
Sun protection & skin care (especially if on photosensitizing meds): reduces skin flares and medication side effects.
Ergonomic changes at work/home: limit pressure on ears (headsets, masks) and reduce exposure to fumes/cold air.
Food safety practices (if immunosuppressed): lowers infection risk from undercooked foods or unpasteurized products.
Regular, team-based follow-up (rheumatology + ENT/pulmonology/ophthalmology): coordinated care improves outcomes, especially when the airway or eyes are involved. Medscape
Core drug treatments
Doses below are typical adult ranges used in practice and literature—your clinician will individualize based on weight, organ involvement, and safety labs. Most drugs are off-label for RP because we lack formal trials and approvals for this rare disease.
Prednisone (systemic corticosteroid) – fast inflammation control
Class: Glucocorticoid. Dose: ~20–60 mg daily for flares; taper to the lowest effective maintenance (often 5–25 mg/d); severe flares can need 80–100 mg/d briefly. Purpose: Rapidly calms active chondritis, airway, eye, or joint flares. Mechanism: Broad suppression of inflammatory gene transcription. Key side effects: Weight gain, high sugar, mood changes, infection risk, bone loss; taper slowly. MedscapeNSAIDs (e.g., ibuprofen, naproxen) – pain and mild inflammation
Class: Nonsteroidal anti-inflammatory. Dose: Ibuprofen 400–800 mg every 6–8 h with food; Naproxen 250–500 mg twice daily. Purpose: Mild auricular/nasal/joint flares. Mechanism: COX inhibition → lower prostaglandins. Side effects: Stomach upset/ulcers, kidney effects, fluid retention.Methotrexate (MTX) – steroid-sparing controller
Class: Conventional DMARD. Dose: 7.5–22.5 mg once weekly (oral or subcutaneous) with folic acid; labs every 4–8 weeks. Purpose: Reduce relapses and lower steroid dose. Mechanism: Anti-inflammatory effects via folate-pathway modulation and adenosine signaling. Side effects: Nausea, mouth sores, liver enzyme rise; avoid in pregnancy; monitor. MedscapeAzathioprine – steroid-sparing alternative
Class: Antimetabolite DMARD. Dose: ~1–2.5 mg/kg/day (often 50–150 mg/d). Purpose: Maintenance control, especially when MTX not suitable. Mechanism: Purine synthesis inhibition → dampened lymphocytes. Side effects: Low blood counts, liver test changes; check TPMT/NUDT15 where available. MedscapeMycophenolate mofetil (MMF) – steroid-sparing option
Class: Antimetabolite DMARD. Dose: 1–1.5 g twice daily (titrate). Purpose: Systemic control, including airway/ocular disease in some series. Mechanism: Inhibits inosine monophosphate dehydrogenase → limits lymphocyte proliferation. Side effects: GI upset, low WBC, infection risk.Cyclophosphamide – for severe, organ-threatening disease
Class: Alkylating immunosuppressant. Dose: Oral ~1–2 mg/kg/day or IV pulse regimens per specialist protocols, then switch to a safer steroid-sparing agent once remission is achieved. Purpose: Rapid control when life- or organ-threatening (e.g., airway collapse, vasculitis). Mechanism: Cytotoxic to proliferating immune cells. Side effects: Infection risk, low blood counts, bladder toxicity—specialist monitoring essential. NCBIDapsone – select patients with milder disease
Class: Anti-inflammatory sulfone. Dose: ~25–200 mg/day; screen for G6PD deficiency first. Purpose: Helpful in some auricular/nasal cases; less favored today. Mechanism: Neutrophil-modulating effects. Side effects: Hemolysis (especially with G6PD deficiency), methemoglobinemia. MedscapeColchicine – adjunct for chondritis/arthritis
Class: Microtubule inhibitor. Dose: 0.6 mg once or twice daily (dose-adjust in kidney disease). Purpose: Some clinicians use it for cartilage/joint flares. Mechanism: Dampens neutrophil function. Side effects: GI upset, drug interactions.TNF-α inhibitors (e.g., infliximab, adalimumab) – for refractory disease
Class: Biologic DMARDs. Typical dosing:
• Infliximab 3–5 mg/kg IV at weeks 0, 2, 6, then every 8 weeks.
• Adalimumab 40 mg subcutaneous every 2 weeks.
Purpose: Used when conventional agents fail or in severe organ involvement. Mechanism: Blocks TNF-α signaling. Side effects: Infection risk (screen for TB/hepatitis), injection/infusion reactions. Evidence: Case series and reviews suggest benefit; infliximab is the most reported TNF agent. MedscapePubMedInterleukin-pathway biologics (tocilizumab, anakinra) and anti-CD20 (rituximab) – targeted options when others fail
Classes & dosing:
• Tocilizumab (IL-6R inhibitor): 8 mg/kg IV every 4 weeks or 162 mg SC weekly/biweekly.
• Anakinra (IL-1 receptor antagonist): 100 mg SC daily.
• Rituximab (anti-CD20): 1000 mg IV on days 1 and 15 (or RA-style 375 mg/m² ×4).
Purpose: Refractory RP, ocular disease, systemic inflammation, or airway disease in series. Mechanisms: Block IL-6 or IL-1 signaling; deplete B cells. Side effects: Infection risk; lab monitoring. Evidence: Retrospective cohorts show response rates to biologics overall around 60% at 6 months, with organ-specific variation. Medscape
Key reality check: RP drug therapy is guided by severity, organ(s) involved, prior responses, and safety. There are no controlled trials, so clinicians blend evidence and experience. Medscape
Advanced” immune therapies
These are specialist-managed and considered after conventional drugs fail or when organs are threatened.
Infliximab (TNF inhibitor) – see dosing above; often the most reported biologic in RP series. PubMed
Adalimumab (TNF inhibitor) – alternative TNF agent with SC dosing convenience. PubMed
Tocilizumab (IL-6 receptor blocker) – helpful in airway/ocular/systemic inflammation in some case series. Medscape
Rituximab (B-cell depletion) – used in refractory cases and certain overlaps; responses are variable. Medscape
Anakinra (IL-1 blockade) – daily SC shots; some rapid responses reported in small series. Medscape
IVIG (intravenous immunoglobulin) – intermittent infusions used as a steroid-sparing adjunct in select refractory cases; exact mechanism is immune modulation. (Dosing regimens vary; specialist protocols apply.)
What about stem-cell or “regenerative” therapy?
Hematopoietic stem-cell transplantation (HSCT) has been tried only in rare, severe, refractory RP, mostly as case reports (including children). Outcomes are inconsistent; relapse can occur, and risks are substantial. This is experimental and considered only in trials or exceptional circumstances. Frontiers
Dietary “molecular” supplements
Evidence in RP itself is sparse. The items below are used for general inflammation control, bone health (especially with steroids), or joint comfort. Always clear supplements with your clinician because of drug interactions and lab monitoring.
Vitamin D3: 800–2000 IU/day typical for bone health (personalize to blood level; respect upper limits). Helps protect bones on steroids; deficiency is common. Office of Dietary Supplements
Calcium: Aim for 1000–1200 mg/day total (diet + supplement) if diet is low; supports bone density while on steroids. (Follow clinician advice if you form kidney stones.) Office of Dietary Supplements
Omega-3 (EPA+DHA): 1–2 g/day combined may lower inflammatory mediators and help joint symptoms; do not exceed ~5 g/day from supplements due to bleeding risk. Office of Dietary Supplements+1
Curcumin (turmeric extract with piperine/optimized forms): 500–1000 mg, 1–2×/day is common in arthritis studies; may ease pain and CRP but interacts with blood thinners. PMC
Glucosamine sulfate: 1500 mg/day (often divided). Evidence for OA pain is mixed; not specific to RP. Use only if clinician agrees. NCCIHMDPI
Chondroitin sulfate: 800–1200 mg/day; mixed evidence in OA; avoid if bleeding risk—discuss first. NCCIH
Magnesium: 200–400 mg/day (if diet is low) supports muscle/bone; excess causes diarrhea; check kidney function. (General nutrition guidance.)
Selenium: 100–200 µg/day if deficient; theoretical antioxidant effects—do not exceed safe upper limits. (General ODS guidance.)
Probiotics: 1–10 billion CFU/day commonly used; may help GI tolerance when on immunosuppressants, but benefits vary; avoid in profound immunosuppression unless your clinician okays it.
Boswellia serrata extract: Doses vary (e.g., 300–500 mg standardized extract 2–3×/day in OA trials); some signal for pain improvement, but data are not RP-specific. PMCPubMed
Surgeries and procedures
Tracheostomy or long-term tracheotomy: creates an airway opening in the neck when upper airway is dangerously narrowed; life-saving in severe laryngotracheal disease. SAGE Journals
Airway stenting (carefully selected cases): rigid or silicone stents hold open collapsible tracheobronchial segments. They can relieve airflow limitation but carry high complication rates (granulation tissue, migration, infection), so centers weigh risks very carefully. PMCERS Publications
Laryngotracheal reconstruction / dilations: endoscopic steroid injection and serial dilations for subglottic stenosis; reconstructive surgery when structure is damaged. Medscape
Nasal reconstruction (septorhinoplasty): repairs saddle-nose deformity after inflammation is controlled; restores function and appearance. Medscape
Cardiovascular surgery: aortic aneurysm repair or valve replacement when large-vessel or valvular inflammation causes dangerous damage. Medscape
Prevention and protection strategies
Don’t smoke; avoid secondhand smoke.
Protect ears and nose from cold, pressure, and trauma.
Use humidified air in dry seasons and during flares.
Vaccinate (timed around immunosuppressants; non-live vaccines preferred).
Treat infections early (especially chest infections).
Keep reflux and chronic cough under control to reduce airway irritation.
Maintain bone health (exercise + vitamin D/calcium as advised) if you need steroids. Office of Dietary Supplements
Track symptoms in a simple diary to spot early relapses.
Use fit-tested, gentle headgear (headsets/masks) that avoid ear pressure.
Keep regular specialist follow-up, even if you feel well.
When to see a doctor—especially urgent care
Breathing trouble, noisy breathing, or rapidly worsening hoarseness.
Severe chest pain, new fainting, or signs of aortic problems.
Red, painful eye with light sensitivity or sudden vision changes.
New hearing loss, vertigo, or severe ear pain with swelling.
High fevers, shakes, or signs of infection while on immunosuppressive medicine.
Persistent weight loss, drenching night sweats, or severe fatigue.
Airway symptoms are always urgent in RP—err on the side of getting help quickly. PMC
Food guidance: “eat this / avoid this” tips
Eat more of:
Fruits and vegetables of many colors—natural antioxidants and fiber.
Fatty fish (salmon, sardines) 2–3×/week for omega-3s.
Whole grains and legumes for slow energy and gut health.
Lean proteins (poultry, tofu, beans, yogurt) to maintain muscle.
Calcium-rich foods (dairy or fortified non-dairy) and vitamin D sources if needed for bone health on steroids. Office of Dietary Supplements
Limit/avoid:
Ultra-processed and high-sugar foods that can fan inflammation.
Excess salt, especially if on steroids (helps control blood pressure and swelling).
Alcohol—avoid entirely with methotrexate and use cautiously with other immunosuppressants.
Grapefruit with drugs that interact (e.g., cyclosporine); always check interactions.
Unpasteurized or undercooked foods if immunosuppressed (food safety matters).
FAQs
Is polychondritis contagious?
No. It is an autoimmune condition—not an infection.Can it be cured?
There is no known cure, but many people achieve long remissions with tailored treatment.Why does my earlobe look normal during a flare?
The earlobe has no cartilage, so it’s spared—this classic sign helps doctors recognize RP. BioMed CentralWill my airway be affected?
Not always. But airway involvement can occur and needs early detection with CT, bronchoscopy, and pulmonary tests if symptoms suggest it. PMCIs there a test that proves RP?
No single blood test proves it. Doctors use clinical patterns and imaging, and sometimes take a biopsy to confirm. MedscapeWhat is tracheobronchomalacia?
A soft, collapsible airway that can narrow when you breathe out. It’s a serious complication of airway-predominant RP. PMCAre there official guidelines?
There are no universal, trial-based guidelines; some countries have practical guidance from expert groups, and treatment is individualized. PMCScienceDirectDo biologics work?
Many patients improve on biologics like infliximab, adalimumab, tocilizumab, rituximab, or anakinra, especially after other drugs fail. Responses vary by organ and person. MedscapePubMedAre there risks with airway stents?
Yes—complications are common, so stents are reserved for carefully selected cases at experienced centers. ERS PublicationsCan diet cure RP?
No. A balanced anti-inflammatory eating pattern supports overall health and bone protection but does not replace medicines.Can I exercise?
Yes—graded, joint-friendly activity is encouraged. Pause and seek help if breathing or chest symptoms worsen.What if I’m pregnant or planning pregnancy?
Several RP medicines are unsafe in pregnancy (e.g., methotrexate, mycophenolate). Plan ahead with your rheumatology/OB team.How long will I need treatment?
RP often needs long-term management; your regimen changes with disease activity and side effects.Could I have VEXAS or another overlap?
A small subset have overlaps such as VEXAS; clues include late-onset systemic inflammation and blood abnormalities. Your specialist will decide if testing is needed. PMCWhat’s the outlook?
Outcomes are improving with earlier recognition and modern immunomodulators; airway and vascular involvement are the biggest determinants, so proactive monitoring is key. Nature
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: August 17, 2025.
