Treatment of relapsing polychondritis: a systematic review
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Treatment of relapsing polychondritis: a systematic review
A. Petitdemange1, C. Sztejkowski2, L. Damian3, T. Martin1, L. Mouthon4, Z. Amoura5,
M. Cutolo6, G.R. Burmester7, J.E. Fonseca8, S. Rednic3, L. Arnaud2
Affiliations: page S5. ABSTRACT the small number of patients treated.
Arthur Petitdemange, MD Objective. Due to the rarity of relaps- While MTX had slightly less efficacy, it
Cédric Sztejkowski, MD ing polychondritis (RP), no randomised is one of the drugs for which data are
Laura Damian, MD, PhD clinical trial has been conducted to the most robust.
Thierry Martin, MD, PhD
Luc Mouthon, MD, PhD date and treatment remains empirical.
Zahir Amoura, MD We performed a systematic literature Introduction
Maurizio Cutolo, MD, PhD review to assess the efficacy of the main Relapsing polychondritis (RP) is a sys-
Gerd R. Burmester, MD, PhD conventional immunosuppressants and temic inflammatory disease primarily
João E. Fonseca, MD, PhD biotherapies used in RP. affecting the cartilaginous structures
Simona Rednic, MD, PhD
Laurent Arnaud, MD, PhD Methods. We searched MEDLINE of the ears, nose and tracheobronchial
Please address correspondence to:
for original articles without language tree, but also the joints, the inner ear,
Pr. Laurent Arnaud, restriction. Abstracts from American the eyes and the cardiovascular system
Service de Rhumatologie, College of Rheumatology (ACR) and among several other systemic mani-
Centre National de Référence des European Alliance of Associations for festations (1). The course of the dis-
Maladies Systémiques et Auto-Immunes Rheumatology (EULAR) were also ease is often unpredictable and varies
Rares Grand-Est Sud-Ouest (RESO),
Hôpital de Hautepierre,
considered for inclusion. Observation- from mild intermittent auricular and/
1 Avenue Molière, BP 83049, al studies and clinical trials reporting or nose chondritis to life-threatening
67098 Strasbourg Cedex, France. on the efficacy of conventional immu- manifestations such as tracheobron-
E-mail: Laurent.arnaud@chru-strasbourg.fr nosuppressants and biotherapies in chial and cardiovascular involvement.
Received on November 2, 2021; accepted adult patients with RP were selected Furthermore, it has recently been dis-
in revised form on January 5, 2022. and pooled response rates for each covered that a subset of patients with
Clin Exp Rheumatol 2022; 40 (Suppl. 134): treatment were computed. RP bears somatic mutations in the gene
S00-S00. Results. Of 304 articles and abstracts encoding for ubiquitin activating en-
© Copyright Clinical and identified, 31 underwent full-text re- zyme 1 (UBA1), which are causal for
Experimental Rheumatology 2022.
view, and 11 were included. The studies the Vacuoles, E1 enzyme, X-linked,
involved a total of 177 patients, exposed Autoinflammatory, Somatic (VEXAS)
Key words: relapsing polychondritis,
to a total of 247 lines of treatments. The syndrome. Patients with VEXAS-RP
systematic review, therapeutics,
main treatments studied (by number are mostly middle-aged or older men
biological therapy, immunosuppressive
of lines) were: TNF inhibitors (TNFi), and are distinguished by frequent he-
agents
n=92; methotrexate (MTX), n=38; toci- matologic abnormalities and increased
lizumab (TCZ), n=26; anakinra (ANA), mortality, among others specific fea-
n=21; rituximab (RTX), n=16; abata- tures (2).
cept (ABT), n=14; cyclophosphamide Since RP is a very rare disease, with a
(CYC), n=14; azathioprine (AZA), prevalence estimated to be as low as a
n=13. The pooled response rates across few cases per million (3), it remains an
studies were: 72% [95% CI: 42-95] for under-researched area. Also, given the
ABT, 66% [95% CI: 49-82] for TCZ, rarity of the disease and the fact that
64% [95% CI: 53-74] for TNFi, 56% the exact cause of RP is still unknown
[95% CI: 37-73] for MTX, 47% [95% (4), the treatment of RP is not stand-
CI: 26-68] for ANA, 43% [95% CI: ardized. Apart from the recent French
20-68] for RTX. Based on more limited therapeutic guidelines (5), no other rec-
data, response rates for AZA and CYC ommendations have been published to
ranged from 38 to 100% and from 25 to date. Patients with mild inflammation
100%, respectively. are usually treated with non-steroidal
Conclusion. In this systematic review anti-inflammatory drugs (NSAIDs)
of available evidence regarding the or low doses of glucocorticoids. Con-
treatment of relapsing polychondritis, versely, patients with severe disease
ABT, TCZ and TNFi were the drugs as- may require high doses of glucocor-
sociated with the best outcomes. ABT ticoids associated with conventional
Competing interests: none declared. efficacy must be interpreted in light of immunosuppressants or biologic ther-
Clinical and Experimental Rheumatology 2022 S-1Treatment of relapsing polychondritis: a systematic review / A. Petitdemange et al.
apies (1). Here, we conducted a sys- Fig. 1. Study flow-chart.
tematic literature review to synthesize
the available evidence on the medical
treatment of RP. The review has been
carried out under the framework of the
European Reference Network on Rare
and Complex Connective Tissue and
Musculoskeletal Diseases (ERN Re-
CONNET).
Material and methods
This systematic review has been per-
formed according to the Preferred Re-
porting Items for Systematic Reviews
and Meta-Analyses (PRISMA) Check-
list 2020.
Literature search and information
sources
We searched MEDLINE from data-
base inception to September 2021 for
original articles without language re-
striction. The search strategy combined
free text search, exploded Medical Data extraction and were included in this systematic re-
Subject Headings (MeSH) terms and The following data were extracted view. One additional article was iden-
synonyms for identifying observation- from each selected study: study design, tified from the reference list of those
al studies and clinical trials which as- number of RP patients, age of patients papers. Also, we further identified
sessed medical treatment of RP in adult at inclusion, gender, RP diagnostic cri- three abstracts which met the eligibil-
patients (see Supplementary document teria used for patient inclusion, dura- ity criteria. The flow chart of the study
S1 for the detailed search strategy). We tion of follow-up, treatments studied is shown in Figure 1.
also searched for additional articles (excluding NSAIDs and glucocorti- The 11 selected studies consisted of
from the reference list of those origi- coids), and outcomes for drug efficacy, eight retrospective observational stud-
nal articles. Finally, we also considered i.e., overall response rates as well as ies, two case series and one open-label
for inclusion abstracts from American partial and complete response rates, non-randomized clinical trial. All but
College of Rheumatology (ACR) since when available. two were published in the 2010s. Two
2009 and from the European Alliance studies focused on specific RP sub-
of Associations for Rheumatology Statistical analyses populations (patients with scleritis and
(EULAR) congress since 2001. For biologic therapies and methotrex- patients with respiratory involvement,
ate, the pooled response rates across respectively).
Study selection and eligibility criteria studies (with their 95% confidence in- The studies involved a total of 177 pa-
Observational studies and clinical trials terval (CI)), weighted according to the tients, with a mean age 50.3 years and
which assessed medical treatment of number of patients in each study, were of whom 62.0% were women. These
RP in adult patients and which report- calculated using the RevMan software patients were exposed to a total of 247
ed the number of responders to each (version 5.3). lines of treatments. Of note, the study
treatment were included. Studies that by Yudoh was not included in the count
exclusively assessed interventional or Levels of evidence and treatment of the total number of patients and
surgical procedures were excluded. recommendation treatment lines because the number of
Single case reports, case series of less The strength of clinical data was grad- patients exposed to each treatment was
than three patients, reviews, editorials ed according to the modified Oxford not reported.
and guidelines were excluded. Arti- Centre for Evidence-Based Medicine The treatments studied were (by de-
cles were selected for inclusion by two Levels of Evidence and Grades of Rec- scending order of lines prescribed):
authors (A.P. & C.S.) independent of ommendation (6). TNF inhibitors (TNFi), n=92 (includ-
each other according to the predefined ing adalimumab [ADA], n=32; inflixi-
inclusion criteria. A first selection was Results mab [IFX], n=27; etanercept [ETN],
performed on the basis of titles and ab- The MEDLINE search yielded 297 ci- n=21; golimumab [GOL], n=3; certoli-
stracts and a second one after reading tations of potential interest, from which zumab [CTZ], n=2; and TNFi without
the full-text articles. seven studies met the inclusion criteria precision, n=7); methotrexate (MTX),
S-2 Clinical and Experimental Rheumatology 2022Treatment of relapsing polychondritis: a systematic review / A. Petitdemange et al. Table I. Characteristics and outcomes of included studies. Author Year Study type Study Diagnostic Number Age at % of Follow-up Treatments studied Outcome, n (%)§ population criteria of inclusion women (median (number of patients (mean ± SD) or mean), treatment lines months administered)* Moulis et al.¶ (7) 2018 Multicentric RP McAdam, 41 46.9±12.5 53.6 6 TNFi (60) R=38 (63.3) CR=14 (23.3) retrospective Damiani IFX (20) R=12 (60) CR=7 (35.0) study & Michet ADA (25) R=16 (64) CR=5 (20.0) ETN (11) R=8 (72.7) CR=0 GOL (3) R=2 (66.7) CR=2 (66.7) CTZ (1) R=0 CR=0 TCZ (17) R=12 (70.6) CR=2 (11.8) ANA (15) R=8 (53.3) CR=2 (13.3) RTX (7) R=5 (71.4) CR=1 (14.3) ABT (6) R=3 (50) CR=1 (16.7) Mathew et al. (8) 2012 Monocentric RP McAdam 43 48.5±20.5 63.6 NA MTX (18)† R=8 (44.4) retrospective study Moulis et al. (9) 2013 Monocentric RP Damiani & 9 44.7 66.7 28 TNFi (14) R=11 (78.6) CR=8 (57.1) retrospective McAdam IFX (2) R=1 (50) CR=1 (50) study ADA (7) R=6 (85.7) CR=4 (57.1) ETN (4) R=4 (100) CR=3 (75) CTZ (1) R=0 CR=0 TCZ (2) R=2 (100) CR=2 (100) ANA (2) R=0 CR=0 ABT (3) R=3 (100) CR=1 (33.3) Leroux et al. (10) 2009 Monocentric RP Michet 9 57.2±9.7 66.7 12 RTX (9)‡ At 6 months (n = 9): At 12 months (n= 6): retrospective R=2 (22.2) R=0 study Stable disease=4 (44.4) Stable disease=2 (33.3) Worsening disease=3 (33.3) Worsening disease=4 (66.7) Pinto et al. (11) 2006 Case series RP NA 6 NA 33.3 NA MTX (2) R=1 (50) LEF (1) R=1 (100) Hoang-Xuan et al. (12) 1990 Case series RP with Damiani 11 52 81.8 36 MTX (2) R=0 scleritis CYC (5) R=5 (100) AZA (3) R=2 (66.7) DAP (8) R=3 (37.5) COL (1) R=0 Dubey et al. (13) 2020 Multicentric RP with Damiani 13 65 69.2 NA MTX (6) R=6 (100) retrospective respiratory AZA (2) R=2 (100) study involvement MMF (2) R=2 (100) CYC (4)** R=1 (25) TNFi (4) R=1 (25) ABT (1) R=1 (100) SCK (1)§§ R=0 (0) Peng et al. (14) 2013 Open-label RP Michet 4 50.5 75 16 ABT (4) R=3 (75)¶¶ prospective study Nakajima et al. 2016 Monocentric RP Damiani 33 NA NA NA MTX (10) R=NA (abstract) (15) retrospective study AZA (8) R=NA CYC (5) R=4 (80) IFX (5) PR=5 (100) TCZ (4) R=1 (33.3)†† Baldini et al. 2013 Monocentric RP NA 8 NA NA NA At 6 months: At 12 months: (abstract) (16) retrospective study TNFi (9) R=NA (12) R=NA (67) ETN (6) R=NA (40) R=NA (60) ANA (4) R=NA (50) R=NA (50) TCZ (3) R=NA (100) NA ‡‡ ‡‡ Yudoh et al. 2010 Retrospective RP NA 239 NA 46.9 NA MTX (NA) R=NA (64) (abstract) (17) declarative study CYC (NA) R=NA (66) CSA (NA) R=NA (74) AZA (NA) R=NA (38) TNFi (13) R=8 (53.8) IFX (10) R=6 (60) ETN (3) R=1 (33) TCZ (3) R=1 (33) ABT: abatacept; ADA: adalimumab; ANA: anakinra; AZA: azathioprine; CNS: central nervous system; COL: colchicine; CSA: cyclosporine A; CTZ: certolizumab; CYC: cyclophosphamide; DAP: dap- sone; ETN: etanercept; GOL: golimumab; IFX: infliximab; LEF: leflunomide; MMF: mycophenolate mofetil; MTX: methotrexate; NA: not available; RP: relapsing polychondritis; RTX: rituximab; SCK: secukinumab; SD: standard deviation; TB: tracheobronchial; TCZ: tocilizumab; TNFi: tumour necrosis factor inhibitors. * A given patient may have received more than one treatment during the study period. § CR: complete response; PR: partial response; R: response (without further detail). ¶ Three patients in this cohort had also been included in Leroux’s analysis. † The number of patients treated and the outcomes for treatments other than MTX were not precisely described. ‡ RTX was given in combination with glucocorticoids (n = 9), MTX (n = 4), MMF (n = 1), AZA (n = 3), and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) (n = 1) (for an associated lym- phoma). ** CYC was only used after failure of conventional DMARDs and was used primarily for tracheobronchomalacia. §§ Secukinumab was started for concomitant ankylosing spondylitis. ¶¶ ABT was effective on chondritis and articular involvement, but two patients presented worsening pulmonary and/or neurological disease leading to treatment discontinuation. †† Outcome was described in only 3 out of 4 patients treated with TCZ. ‡‡ As reported in the abstract. Clinical and Experimental Rheumatology 2022 S-3
Treatment of relapsing polychondritis: a systematic review / A. Petitdemange et al.
n=38; tocilizumab (TCZ), n=26; anak- the most effective. In the study by roughly similar, the choice of one over
inra (ANA), n=21; rituximab (RTX), Moulis et al. (7), the high overall re- another should take into account the
n=16; abatacept (ABT), n=14; cyclo- sponse rate of ETN must be balanced disease phenotype (e.g., MTX may be
phosphamide (CYC), n=14; azathio- by a high discontinuation rate for lack preferred in case of joint involvement),
prine (AZA), n=13; dapsone (DAP), of efficacy. AZA showed overall re- the patient’s comorbidities and pref-
n=8; mycophenolate mofetil (MMF), sponse rates ranging from 38 to 100% erences, the tolerance profile of each
n=2; and colchicine (COL), lefluno- and CYC from 25 to 100%. In the study molecule, as well as cost-effectiveness
mide (LEF) and secukinumab (SCK) by Dubey et al. (13), the poor outcome considerations. The combination of
(initiated for concomitant ankylosing observed with CYC may be explained MTX with one of these biotherapies
spondylitis), with one each. by its use in refractory disease. Too few may also be considered in order to
The pooled response rates across stud- patients were exposed to other mol- improve treatment efficacy and/or to
ies were: 72% [95% CI: 42-95] for ecules to draw any conclusions. avoid the development of antidrug
ABT, 66% [95% CI: 49-82] for TCZ, It should be noted that, for a given antibodies. It should be noted that the
64% [95% CI: 53-74] for TNFi (IFX, drug, response rates were highly vari- use of CYC may be preferable in rap-
59% [95% CI: 42-75]), 56% [95% CI: able between the different studies. This idly life-threatening forms to achieve a
37-73] for MTX, 47% [95% CI: 26-68] may be due to differences in response prompt therapeutic response. ABT, on
for ANA, 43% [95% CI: 20-68] for definition, in the timing of response the other hand, appears to be a good
RTX. evaluation as well as in disease sever- option in case of nasal or auricular
The detailed characteristics and out- ity. chondritis or articular involvement but
comes of the included studies are re- This review highlights the paucity of should be used with caution in case
ported in Table I. available evidence on the treatment of respiratory or neurological disease
of RP. Indeed, our literature search given the results from Peng et al. (14).
Discussion identified only 11 studies, of which Of note, the management of VEXAS-
In this systematic review of 11 studies, all but one were retrospective studies RP patients should be considered sepa-
we assessed the efficacy of the main with low levels of evidence, not ex- rately, as these patients often present
conventional immunosuppressants and ceeding 2b. To date, no randomised refractory disease with corticodepend-
biologic therapies used in RP, based on clinical trial has been conducted in RP. ency (18). However, there is currently
available evidence to date. This has hampered the development no published data regarding the opti-
According to the summary response of evidence-based recommendations mal treatment of this particular popula-
rates across studies, ABT, TCZ and to guide treatment, which is therefore tion and studies investigating this issue
TNFi were the drugs associated with essentially based on data provided are particularly needed.
the best outcomes, with mean response by case reports and small case series. This study has some limitations. The
rates of 72%, 66% and 64%, respec- Minor nasal or auricular chondritis definition of the terms ‘response’, ‘par-
tively. However, caution is particularly or joint involvement may respond to tial response’ and ‘complete response’
needed regarding ABT given the small NSAIDs, glucocorticoids, colchicine was not systematically provided and
number of patients treated. Further- or dapsone. Conventional and biologi- may have varied between studies.
more, in the study by Peng et al. (14) cal disease-modifying antirheumatic Likewise, the characteristics of the dif-
while abatacept was effective on chon- drugs (DMARDs) are used as glucor- ferent study populations differed. This
dritis and joint involvement, worsening ticoid-sparing agents or in cases of heterogeneity limits the relevance of
of respiratory and neurological disease more severe disease. However, due to pooled analyses and of comparisons
was observed in two out of four pa- the aforementioned issues, the choice between studies. Moreover, the assess-
tients. MTX had a pooled response rate of the DMARD to use remains largely ment of drug efficacy could have been
of 56% while ANA and RTX showed empirical. The strength of our study is refined by analysing information such
lower efficacy, with mean response to synthesize data from about 250 treat- as glucocorticoid-sparing effect or per-
rates of 47% and 43%, respectively. ment lines, thereby providing a level of sistence of treatment. However, these
Of note, in the study by Leroux (10), evidence that has never been achieved data were too inconsistently reported to
no complete remission was observed before and addressing one of the ma- be relevant for inclusion in our review.
with RTX and seven out of nine pa- jor unmet needs in the field of RP (1). The same was true for safety data,
tients experienced worsening disease It suggests that IFX, ADA, TCZ and which were only rarely reported. Final-
whereas good outcomes were reported MTX are the most effective DMARDs ly, as most studies include indistinctly
in the study by Moulis et al. (7). Such to treat RP as well as those for which different phenotypes of the disease,
a difference could be attributed to the the data are the most robust. Thus, whether in terms of severity or organ
recruitment of patients with more se- when the severity of the disease or cor- involvement, and as response was most
vere disease in the study by Leroux et ticodependency warrants the initiation often not specified by organ involve-
al. (10). Among TNFi, IFX and ADA of a DMARD, we would suggest the ment, it was not possible to stratify the
are both those for which we have the use of one of the four abovementioned response rates on disease phenotype.
most data and those that appear to be drugs. As their efficacy appears to be In conclusion, our systematic review
S-4 Clinical and Experimental Rheumatology 2022Treatment of relapsing polychondritis: a systematic review / A. Petitdemange et al.
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