Refractory relapsing polychondritis: challenges and solutions

Figures & data

Table 1 Systemic vasculitis and connective tissue disorders associated with relapsing polychondritis (not exhaustive)

Systemic vasculitis	Behçet’s disease (MAGIC syndrome), granulomatosis with polyangiitis, polyarthritis nodosa, Churg and Strauss’s syndrome
Connective tissue diseases	Sjögren’s syndrome, rheumatoid arthritis, systemic lupus erythematosus, mixed connective tissue diseases, systemic sclerosis
Spondyloarthritis	Ankylosing spondylitis, psoriatic arthritis, reactive arthritis, inflammatory bowels diseases
Other autoimmune diseases	Autoimmune thyroiditis, type 1 diabetes, familial Mediterranean fever, myasthenia gravis, primary biliary cirrhosis
Malignancies	Myelodysplastic syndromes, lymphomas, myeloproliferative neoplasms, thymoma
Dermatosis	Sweet’s syndrome, neutrophilic dermatosis, pyoderma gangrenosum, leukocytoclastic vasculitis, psoriasis

Abbreviation: MAGIC, mouth and genital ulcers with inflamed cartilage

Figure 1 Relapsing polychondritis: auricular chondritis during acute phase (A); chronic phase with collapse of the cartilage of the upper pole of the ear (B); saddle nose deformity (C); episcleritis (D).

Note: Reproduced with permission from Club Rhumatismes et Inflammations. [website on the Internet]. Available from http://www.cri-net.com/autres-projets/base_images/display_rub.asp?rub=poly_atro.⁷⁹

Table 2 Comparison of demographics and clinical features of 10 cohorts around the world

	McAdam et al^Citation8 (USA)	Michet et al^Citation10 (USA)	Zeuner et al^Citation25 (Germany)	Trentham and Le^5* (USA)	Mathew et al^Citation17 (USA)	Sharma et al^Citation3 (India)	Shimizu et al^Citation42 (Japan)	Lin et al^Citation12 (China)	Dion et al^Citation13 (France)	Pallo et al^Citation54 (Brazil)
Number of patients (n)	159	112	62	66	43	26	239	158	142	30
Mean age at diagnosis (years)	44	51	46	46	44	45	53	45	43	49
Female/male (n)	76/83	55/57	26/63	49/17	23/20	16/10	112/127	63/95	86/56	21/9
Mean follow-up (months)	NR	72	12	96	84	NR	64	NR	156	72
Auricular chondritis (%)	89	85	93	95	88	96	78	68	89	100
Hearing impairment (%)	46	30	19	42	37	42	27	25	27	30
Laryngotracheal involvement (%)	56	48	30	67	37	11	50	69	43	57
Nasal chondritis (%)	NR	29	56	20	35	81	39	54	63	10
Arthritis (%)	81	52	53	85	60	54	39	18	69	60
Ocular involvement (%)	65	51	50	57	57	42	46	44	56	37
Cardiovascular involvement (%)	9	6	22	8	27	11	7	10	22	3
Skin involvement (%)	17	28	24	38	NR	26	14	46	29	NR

Note:

* Based on 36 patients with relapsing polychondritis and 30 patients from literature review.

Abbreviation: NR, not recorded.

Table 3 Efficacy of biologics in relapsing polychondritis: 2017 update (preliminary reports)

	Yes		Partial		No		Total
	2012	2017	2012	2017	2012	2017	2012	2017
Infliximab	14	26	4	16	13	15	31	57
Etanercept	5	7	0	9	3	3	8	19
Adalimumab	2	12	0	17	2	3	4	32
Golimumab	0	2	0	2	0	0	0	2
Certolizumab	0	1	0	0	0	0	0	1
Rituximab	0	2	2	7	9	9	11	18
Anakinra	4	9	0	8	1	1	5	18
Tocilizumab	2	14	0	14	0	0	2	30
Abatacept	1	3	0	4	0	1	1	8

Club Rhumatismes et Inflammations. [website on the Internet] Available from http://www.cri-net.com/autres-projets/base_images/display_rub.asp?rub=poly_atroAccessed December 22, 2017

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