Respiratory subtype of Relapsing Polychondritis
(RP) frequently presents as difficult asthma: a
descriptive study of respiratory involvement in RP
with 13 patients from a single UK centre
Shirish Dubey, Colin Gelder, Grace Pink, Asad Ali, Christopher Taylor, Joanna Shakespeare, Susan
Townsend, Patrick Murphy, Nicholas Hart, David D'Cruz
Please cite this article as: Dubey S, Gelder C, Pink G, et al. Respiratory subtype of Relapsing
Polychondritis (RP) frequently presents as difficult asthma: a descriptive study of respiratory
involvement in RP with 13 patients from a single UK centre. ERJ Open Res 2021; in press
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Respiratory subtype of Relapsing Polychondritis (RP) frequently presents
as difficult asthma: a descriptive study of respiratory involvement in RP
with 13 patients from a single UK centre
Dr Shirish Dubey MBBS, FRCP, FRCPI, M.Med.Ed
Consultant Rheumatologist, Nuffield Orthopaedic Centre, Oxford University
Hospitals NHS Trust, Windmill Road, Oxford OX3 7LD
University Hospital Coventry and Warwickshire NHS Trust, Coventry CV2 2DX
Dr Colin Gelder
Consultant Respiratory Physician (retired), University Hospital Coventry and
Warwickshire NHS Trust, Coventry CV2 2DX.
Dr Grace Pink
Fellow in Respiratory Medicine, University Hospital Coventry and Warwickshire
NHS Trust, Coventry CV2 2DX.
Dr Asad Ali
Consultant Respiratory Physician, University Hospital Coventry and
Warwickshire NHS Trust, Coventry CV2 2DX.
Dr Christopher Taylor
Consultant Respiratory Physician, Heart of England NHS Foundation Trust,
Birmingham. B9 5SS.
Mrs Joanna Shakespeare MSc, BSc.
Clinical Scientist, Department of Respiratory and Sleep Sciences, University
Hospitals Coventry and Warwickshire NHS Trust, Coventry, CV2 2DX
Mrs Susan Townsend
Respiratory Clinical Nurse Specialist, Department of Respiratory and Sleep
Sciences, University Hospitals Coventry and Warwickshire NHS Trust, Coventry,
Dr Patrick Murphy
Consultant Respiratory Physician, The Lane Fox Unit, St Thomas’ Hospital
London SE1 7EH
Professor Nicholas Hart MB BS BSc PhD MRCP FFICM
Professor of Respiratory & Critical Care Medicine, Clinical Director Sleep,
Respiratory and Critical Care, Guy's and St Thomas' NHS Foundation Trust,
London SE1 7EH
Professor David D’Cruz MD, FRCP.
Consultant Rheumatologist, Louise Coote Lupus Unit, Guy’s Hospital, London SE1
Corresponding author: Dr Shirish Dubey.
All authors have contributed to the study design and write up. SD, GP, CD,
AA, JS and ST have helped with data collection and data analysis.
Conflict of information and disclosure forms attached for all authors.
The article fulfils all requirements of the Strobe checklist.
Relapsing polychondritis (RP) was described by Pearson et al. in 1960  as a rare
multisystem disease characterised by recurrent episodes of inflammation and
subsequent degeneration of cartilage and connective tissue throughout the body. RP
most commonly affects the respiratory tract, nose, ears and joints [1-4]. McAdam et al
described 6 classical features of relapsing polychondritis, namely bilateral auricular
chondritis, nasal chondritis, respiratory tract chondritis, seronegative inflammatory
arthritis, ocular inflammation and audiovestibular damage . McAdam’s diagnostic
criteria needed 3 out of 6 of the aforementioned clinical features for confirmation of
diagnosis. Additional diagnostic criteria were developed by Damiani and Michet
[2,5]. Both these groups have developed A and B criteria with Damiani criteria
keeping all 6 primary clinical features as A criteria and additionally including
histological confirmation as a B criterion and response to corticosteroids or Dapsone
as a C criterion. 3 A criteria or 1 A and B, or 2 A with C are needed for diagnosis.
Michet criteria include nasal, auricular and laryngotracheal cartilage inflammation as
A criteria with the rest as B criteria, and 2 A or 1 A and 2 B criteria are needed for
Respiratory tract chondritis is thought to affect up to 50% of patients during the
course of their disease [2,3,6] and remains the primary cause of mortality in RP .
Patients often experience airway symptoms such as dyspnoea, cough, chest
discomfort, hoarseness, stridor  and even complete aphonia in some cases  due
to inflammatory oedema of the larynx, trachea and bronchi. The underlying chronic
cartilage inflammation in the tracheobronchial tree leads to tracheomalacia ; or
tracheobronchomalacia (TBM) when this extends to one or both primary bronchi.
Both phenomena can result in exaggerated airway narrowing during expiration and
widening during inspiration [9-11], demonstrable in pulmonary function tests and
computerised tomography (CT) scans of the chest. Unless early diagnosis and
appropriate medical or surgical interventions are in place, the progressive cartilage
destruction in the airways due to recurrent cartilaginous inflammation may ultimately
result in life-threatening airway obstruction and dynamic airway collapse . RP can
involve the eyes, neurological system, heart and blood vessels and there is an
association with HLA DR4 allele . Respiratory problems can be particularly
difficult to treat, and very little data exist to guide us with regards to optimal
screening and assessment modalities for tracheomalacia or tracheobronchomalacia
(TBM). Management of these patients continues to remain a challenge and the
diagnostic delay can often result in significant damage, which necessitates long term
mechanical support through stents or pneumatic support through continuous positive
airway pressure (CPAP) [14, 15]. Despite best treatment, patients are often left with
substantial life changing disability.
We describe a series of patients with RP all of whom had respiratory involvement.
Most had presented to respiratory clinics or had been admitted to hospital with severe
shortness of breath. All patients attended University Hospital Coventry and
Warwickshire NHS Trust which is based in Coventry in West Midlands in UK, and is
a secondary care provider for a population of around 500,000. This case series
describes the respiratory manifestations and aims to increase the awareness of RP in
patients presenting with respiratory symptoms, particularly in individuals who appear
to have oral corticosteroid dependent asthma.
Materials and Methods
We reviewed the medical records of thirteen patients with relapsing polychondritis;
all of whom had respiratory involvement. Patients were identified through the
respiratory and rheumatology clinics at a single centre between 2013 and 2018 and
patients were often seen together in a combined clinic. The diagnosis of RP was made
clinically using the clinical diagnostic criteria [2,3,5]. Disease activity was assessed
using Relapsing Polychondritis Disease Activity Index (RPDAI) which includes
scoring on each organ that can be affected by RP as well as C-Reactive Protein (CRP)
. There are 28 different items with scores ranging from 1 to 24. Respiratory
chondritis scores 14 without and 24 with respiratory failure and is the highest scoring
item in RPDAI. Patients’ demographic characteristics, clinical features, diagnostic
test results and therapeutic interventions were noted. The database was set up in 2016
and details of patients were updated regularly. Ethical approval was obtained from
Research and Development office within our Trust; approval number – GF 0267.
Statistics are predominantly descriptive, and MS Excel programme was used to
assimilate the data.
We identified 13 patients with relapsing polychondritis, all of these patients had
respiratory involvement. We did not need to exclude any patients due to lack of
respiratory involvement. Most of these patients (10 out of 13) were identified in
‘difficult asthma’ clinics with 2 being diagnosed following an inpatient admission
with acute shortness of breath and 1 diagnosed from a rheumatology clinic. The
demographics are described in table 1. Male to female ratio was 1:3 with 3 males and
9 females. The median age of the patients was 65 (range 28 to 76) years. Most
patients had other co-morbidities with diabetes being the commonest in 5 patients and
hypertension seen in 4 patients. Other auto-immune disorders were diagnosed in 7 of
these patients. Psoriasis and hypothyroidism were noted in 2 patients each. One
patient had overlap with Behcet’s disease (mouth and genital ulcers with inflamed
cartilage - MAGIC syndrome), another had ankylosing spondylitis. [Table 1, figures
We found that 8 patients (62%) had bilateral auricular chondritis and nasal chondritis,
whilst 10 patients (77%) had seronegative polyarthropathy with 2 patients (15%)
having ocular inflammation and 5 patients (38%) had audiovestibular damage [Figure
6]. All patients had good response to oral Prednisolone and fulfilled criteria for
diagnosis of RP (Damiani). Most patients (10 out of 13) were picked up from the
difficult asthma clinics. All patients had wheeze and persistent cough and hence a
diagnostic label of asthma, but it was the presence of monophonic wheeze, presence
of inspiratory stridor in 2 patients, barking nature of cough in 2 patients and lack of
classical reversibility and response to steroids that led to the suspicion of underlying
more complex airway issues and possible expiratory airway collapsibility. Patients
with good response to oral prednisolone demonstrated return of their signs and
worsening of other symptoms with dosage reduction below 20mg daily leading to
further suspicion about the underlying diagnosis. Dynamic CT (inspiratory and
expiratory) images were obtained along with flexible bronchoscopy. Bronchoscopy
was performed in 4 patients. Mild sedation using intravenous midazolam and local
analgesia with 2% lignocaine was instilled. Patients were able to co-operate and
forcibly exhale. Views were taken from the proximal and distal trachea, right and left
main bronchi and segmental bronchi during inspiration and forced expiration. 50% or
more reduction in the cross sectional area of the airway during the dynamic
bronchoscopy and CT were used as the diagnostic cut off for the diagnosis of TBM.
Two of our patients demonstrated smooth thickening of the airway wall and luminal
narrowing of the distal trachea and main bronchi and one demonstrated symmetrical
stenosis of the large airways, whereas the remaining had >50% reduction of the
airway luminal area with crescentic appearance of the airway due to flattening of
airway walls during expiration.
Although other features such as bilateral auricular chondritis or nasal chondritis had
been present in 8 patients, they had rarely complained about these symptoms to their
clinicians as other symptoms, particularly severe breathlessness were their primary
concern. Eliciting these symptoms required direct questioning. One patient had
classical nasal bridge collapse which they previously told several clinicians (via
interpreters) was the result of childhood trauma, although on detailed questioning
there was in fact no history of trauma. Seronegative inflammatory arthritis was a
presenting feature in 2 patients (predominantly large joints), and had been noted in 10
Laboratory testing showed anaemia in seven patients and raised inflammatory
markers including CRP or erythrocyte sedimentation rate in 6 patients. As a number
of patients were on long term corticosteroids for ‘difficult asthma’, it was difficult to
get accurate trends of inflammatory markers prior to treatment. None of the patients
had evidence of eosinophilia at any point. Rheumatoid factor, anti-cyclic citrullinated
antibodies, antinuclear antibodies, anti-double stranded DNA antibodies and
neutrophil cytoplasmic antibodies were all negative, although one patient had
antiphospholipid antibodies. Chest radiographs were normal in 11 patients, 2 had
shown features of pleural effusions and these were confirmed on CT scans later. None
of the patients had any other features to suggest ANCA associated vasculitis.
In 12 out of 13 patients, flow-volume loops demonstrated flattening of either
inspiratory or expiratory curves, or both. Flattening of the expiratory limbs in flow-
volume loops was prevalent in most, suggesting large airway collapsibility during
expiration. [Figures 7,8]. There was no evidence of reversibility with beta 2 agonists
in 11 patients, whilst 1 patient with small airway disease showed reversibility with
likely co-existent asthma.
Treatment: Corticosteroids were used in all patients, and disease modifying anti
rheumatic drugs (DMARDs) such as Methotrexate 15 to 25 mg weekly (6 patients),
Azathioprine 1-2.5 mg/kg/d (2 patients) and Mycophenolate mofetil 1-2 grams daily
(2 patients) were successful in reducing disease activity (Table 2). One patient
developed hypogammaglobulinemia which was thought to be secondary to
immunosuppression and was treated with replacement IV Immunoglobulin (IVIG) as
she was having recurrent infections (predominantly chest infections). Prednisolone
was usually started at 1 mg/kg/day orally in patients with respiratory failure and 0.5
mg/kg/day in patients without respiratory failure with gradual tapering every 2-4
weeks initially. Dose reduction was achieved in all cases but 4 patients struggled to
wean Prednisolone dose down below 10 mg. In 2 patients, we only used <20 mg
Prednisolone, higher doses were not needed. IV Cyclophosphamide was used in 4
cases, but was thought to be unsuccessful in 3 of these on the basis of lack of
symptomatic benefit. Cyclophosphamide was only used after failure of conventional
DMARDs and was used primarily for TBM. Patients with severe airway collapse
>90% of airway area with disabling symptoms were considered for large airway
stenting alongside medical therapies. Successful stenting was performed in 3 patients;
in one other patient the stent had to be removed as it was exacerbating infections and
another due to continuous coughing. Six patients with moderately severe airway
compromise (75%-90%) and significant breathlessness on exertion were receiving
intermittent ambulatory CPAP, 2 discontinued due to lack of tolerance. NIV was used
with maximum inspiratory pressure (IPAP) of 24 and expiratory pressures (EPAP) of
10cm H2O whilst CPAP pressures were between 10 and 13 cm H2O. Overnight sleep
studies excluded significant sleep disordered breathing in these patients. Within this
cohort, 7 patients have had recurrent admissions for ‘flare of asthma’ prior to the
diagnosis with 3 of these not requiring further inpatient admissions once
immunosuppression was instituted. [Table 2 here]
Biological DMARDs were tried in 4 patients with anti-TNF therapies being
successful in 1 and unsuccessful in 3 patients (two due to inefficacy, another due to
allergic reactions to both Etanercept and Adalimumab). Of the 3 patients who failed
anti-TNF therapy, 2 were tried on other agents, with one patient responding well to
Abatacept whilst another patient was started on Secukinumab for ankylosing
spondylitis and had good response for spinal disease, but no change in RPDAI. Eight
patients are still under regular follow-up (FU) and have been under FU for more than
5 years since diagnosis, one has been lost to follow up and 4 patients have died. In
two of these cases, primary cause of death was chest infection, in the other two, it was
unrelated causes, one from complications of myelodysplasia.
A number of studies have described small numbers of patients with respiratory
features and some have shown airway involvement to be the leading cause of death in
RP [1,3,6,15,17]. TBM has been reported in literature in up to 50% of patients with
RP. Our series saw TBM as the commonest presentation of RP, although it is quite
likely that a number of patients with less serious problems might not have been
appropriately diagnosed given the rarity of this condition. A French series reported
142 patients with RP who formed three distinct patterns – haematological, respiratory
and ‘mild’ phenotypes . Within the respiratory phenotype which formed 22.5% of
their series, auricular involvement was less common, something we have seen as well.
Similar to our series, they found that these patients received more intensive
treatment, were prone to infections, and were frequently admitted to the ICU.
Our series provides more detail about the respiratory sub-type of RP with specific
focus on presentation and management. We did not need to exclude any patients with
RP due to lack of respiratory involvement. Given its rarity, it is likely that there are
other patients with less serious manifestations that have not reached rheumatology or
respiratory clinics and have not been given the diagnosis yet. The majority of these
patients were originally thought to have oral corticosteroid dependant asthma, and
once TBM was suspected or diagnosed, physicians started searching for and finding
other features of RP. Patients had not complained about the other manifestations such
as chronic auricular chondritis or nasal chondritis as the symptom of breathlessness
Physical treatments of TBM with stenting and CPAP are well recognised [6-9,17,19],
however, there is very little information in literature about pharmacological treatment
of TBM through immunosuppression. This is important as TBM can be the only
manifestation of RP . In our series, most patients had responded well to
pharmacological therapy, although some needed stent insertion to support the
bronchial tree. Stenting also had mixed results and it is unclear as to whether there are
specific features that would indicate use of stents in preference to drug therapy.
Stenting is most likely to be useful after optimal control of active disease (to stabilise
damaged section of the tracheobronchial tree once medical treatment has controlled
active inflammation). Complications following stenting are relatively common with
one study showing 49/58 patients having a complication commonest of which are
stent migration, infection and partial obstruction . Aggressive early management
can be difficult to achieve when the patient has been symptomatic for so many years
and airway damage has accumulated before the diagnosis is made. Intermittent
ambulatory continuous positive airway pressure has been described previously with
variable results [22-24]; our group has previously described successful use of CPAP
in TBM. Such long term use of portable NIV combined with overnight CPAP has not
been reported to our knowledge. We have seen good symptomatic improvement and
long term stability with CPAP used in this fashion together with medical treatments.
Clinical and symptomatic evaluation, dynamic (inspiratory and expiratory) CT scans
and flexible bronchoscopy were critical in establishing the diagnosis of TBM which is
consistent with reports from literature [8-10]. Other features of RP were identified
clinically although recent reports suggest Positron Emission Tomography (PET CT)
might be an additional resource for defining the severity and extent of disease .
PET CT has other potential advantages as it can a) differentiate damage from active
inflammation, and b) provide information about large vessel vasculitis and other
organs that are not easy to assess clinically. We have not used this modality in our
patients, and this can be evaluated in future studies.
The prevalence of relapsing polychondritis in Coventry appears to be at least 26 per
million on the basis that we have 13 patients locally within our catchment area of
around 500,000. If these prevalence data were true for the rest of UK as well, one
would expect roughly 1500 additional patients! It is difficult to estimate the true
prevalence for a rare condition, and the literature has offered very wide estimates
(between 3.5 per million to 23 per million). Hungarian data suggest similar numbers
(23 per million) to the numbers estimated here based on 233 cases from a population
of 10 million . Incidence in that study was around 3.5 per million patient years.
Incidence of RP in a UK study was 0.71 per million patient years and prevalence was
estimated at 9 per million . In Rochester (Minnesota), the incidence of RP was
estimated at 3.5 per million . Given the rarity of the condition and difficulty in
diagnosis, it is not a surprise that there is such wide variation. This study provides
new impetus to looking for specific features of RP which may have a major influence
on incidence and prevalence estimates.
There are no controlled clinical trials in this area (as is the case for a number of rare
diseases), and it may be possible to set up trials in this area if the prevalence is
significantly higher than was previously thought. There is a need to increase
awareness of this disease amongst all the specialties that are likely to come across
these patients. Optimal management of these patients continues to remain a challenge.
The exact pathogenesis is not clearly understood. Various immune processes that
have been described include reduction of immunoregulatory cells, antibodies
attacking cartilage tissue elements like type-II, type-IX, and type-XI collagen and
matrilin1, changes in cytokine profiles, deposition of immune complexes, and
insufficient tissue regeneration [28-34]. This makes it quite challenging when
choosing drugs for refractory patients. Within our cohort, we observed some
responses to DMARDs with Methotrexate, Azathioprine and Mycophenolate being
successful. In fact, in one patient we were able to completely stop corticosteroids and
have not needed to go back to corticosteroids for more than 2 years. Responses to
biological agents and intravenous (IV) Cyclophosphamide have been modest in this
cohort – this may be due to delay in diagnosis which can sometimes be a number of
years. Also, we have not routinely used IV Cyclophosphamide for induction, but
tended to use it when other agents have failed. Disease activity and damage scores
have been developed [16, 35] and are of use in documenting response to treatment;
and also serve as a reminder of the various manifestations of this rare illness. Multiple
biological agents have been tried, but due to the rarity of the condition, there are no
randomised controlled trials in this field. A French national study looking at biologics
in RP did not demonstrate any clear trends that would help guide use of biological
This is a retrospective review and studies of this sort are subject to systemic biases
which are applicable to this study. Prevalence data are affected by referral pathways
and other biases which would be applicable to this study. Also patients presenting
with respiratory symptoms were selected so this is a referral bias. There is also likely
to be left censorship bias as some patients who may have died or were lost to follow-
up would not have been included.
Relapsing Polychondritis, although rare, with prevalent respiratory involvement may
be the cause of significant morbidity and mortality. Patients may be misdiagnosed
with other respiratory diseases in particular being labelled as ‘difficult asthma’.
There is an important need to recognise and diagnose relapsing polychondritis, as
there are specific treatment options including DMARDs that these patients are likely
to benefit from. Awareness of this condition is crucial to enable early diagnosis and
clinical interventions to reduce the risk of life threatening airway collapse.
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Table 1: Clinical features of patients with Relapsing polychondritis.
HTN, T2DM, OA
HTN, Angina, AF,
HTN, obesity, acoustic
T2DM, obesity, MI,
AF, CKD, dementia,
T2DM, IHD, CKD,
BAC: Bilateral auricular chondritis, NC: Nasal chondritis, RTC: Respiratory tract chondritis,
SP: seronegative polyarthritis, OI: Ocular inflammation, AD: Audiovestibular damage.
T2DM: Type 2 Diabetes mellitus, HTN: Hypertension, TB: Tuberculosis, OA: Ost eoarthritis,
AF: Atrial fibrillation, MI : Myocardial infarction, CKD : Chronic kidney disease, COPD:
Chronic obstructive pulmonary disease.
Table 2: Pharmacological and non pharmacological treatment for patients with RP
Pred 10 mg
ADA and ETN
SSZ, HCQ, ABT,
Pred 7.5 mg
Pred 10 mg
Pred 10 mg
Pred 5 mg
Pred 5 mg
MTX, AZA, HCQ
Pred 10 mg
MTX, ETN, HCQ,
Pred 5 mg
Pred 5 mg
IS: Immunosuppressant drugs, MTX: Methotrexate, MMF: Mycophenolate mofetil, SSZ: Sulfasalazine, HCQ:
Hydroxychloroquine, ADA: Adalimumab, AZA: Azathioprine, ABT: Abatacept; IVIG: Intravenous
immunoglobulin, ETN: Etanercept, Pred: Prednisolone, HCT: Hydrocortisone, RPDAI: Relapsing
Polychondritis Activity Index.
A: Admission CT scan showing near complete collapse of trachea in a patient that was subsequently diagnosed with RP.
B: Repeat CT after IV corticosteroids with inspiratory and expiratory films showing significant improvement of tracheal narrowing (expiratory
A: Another patient with admission CT chest showing near complete collapse of trachea and pleural effusions
B: Repeat CT after treatment with high dose corticosteroids with improvement in trachea and resolution of pleural effusions.
Figure 3: Another patient with collapse of trachea.
A: Another patient with presentation CT showing significant narrowing of trachea
B: Post treatment imaging showing improvement in dimensions of trachea.
Figure 5: Pre-treatment tracheal collapse in another patient
Figure 6: Patient images demonstrating auricular chondritis with inflammation of the external ear with sparing of non-cartilaginous part.
Figure 7: Flow volume loop of patient 4 showing flattening of the expiratory limb and inspiratory limb to a lesser extent
Figure 8: Flow-volume curve of patient 6 showing flattening of the expiratory limb and inspiratory limb to a lesser extent.