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Arthritis Rheumatol. 2015 Nov;67(11):3027-36. doi: 10.1002/art.39292.

Musculoskeletal symptoms in patients with cryopyrin-associated periodic syndromes: a large database study.

Author information

1
Morvan University Hospital, Brest, France.
2
Nord-de-France University and Claude-Huriez University Hospital, Lille, France.
3
Kremlin Bicêtre University Hospital, Le Kremlin Bicêtre, France.
4
Necker-Enfants Malades University Hospital and Paris Descartes University, Paris, France.
5
Arnaud de Villeneuve University Hospital, Montpellier, France.
6
Yves le Foll Hospital, Saint-Brieuc, France.
7
Tenon University Hospital, AP-HP, and Paris VI Pierre et Marie Curie University, Paris, France.
8
Hôtel Dieu University Hospital, Nantes, France.
9
Necker-Enfants Malades University Hospital, AP-HP, and INSERM U768 Laboratory, Paris, France.
10
Rouen University Hospital, Rouen, France.
11
Pellegrin-Enfants University Hospital, Bordeaux, France.
12
University Hospital, Nancy, France.
13
Hautepierre University Hospital, Strasbourg, France.
14
Women-Mothers-Children University Hospital, Lyon, France.
15
Montpellier University Hospital, Montpellier, France.
16
Hotel Dieu Hospital, Paris, France.
17
Jean Verdier University Hospital, AP-HP, Paris XIII University, Paris, France.
18
Dupuytren University Hospital, Limoges, France.
19
Reims University Hospital, Reims, France.
20
Rennes University Hospital, Rennes, France.
21
Nord University Hospital, Saint-Etienne, France.
22
Saint Chamond, France.
23
North University Hospital, AP-HM, Marseille, France.
24
Saint André Hospital, and Bordeaux University Hospital, Bordeaux, France.
25
North University Hospital, Amiens, France.
26
Tenon University Hospital, AP-HP, Paris, France.
27
Saint Etienne University Hospital, Saint-Etienne, France.
28
Nîmes University Hospital, Nîmes, France.
29
Claude Huriez University Hospital, Lille, France.
30
Toulouse University Hospital, Toulouse, France.
31
Cochin Institute and University Hospital, AP-HP, Paris Descartes University, Paris, France.
32
La Cavale Blanche University Hospital, Brest and EA2216 Brittany University (UBO), Brest, France.

Abstract

OBJECTIVE:

To determine the type and frequency of musculoskeletal symptoms at onset and during followup of cryopyrin-associated periodic syndromes (CAPS).

METHODS:

We retrospectively recorded the articular and muscular symptoms of patients with CAPS followed up in French hospitals. Data were presented as frequencies or the median (range), and patient groups were compared using chi-square test, Fisher's exact test, and Mann-Whitney test.

RESULTS:

The study included 133 patients (33 children), 20 with familial cold autoinflammatory syndrome, 88 with Muckle-Wells syndrome, 22 with chronic infantile neurologic, cutaneous, articular syndrome, and 3 with unclassified CAPS. The median age was 35 years (range 0-78 years) at the time of the study, 1 year (range 0-41 years) at symptom onset, and 23 years (range 0-58 years) at diagnosis. The disease was sporadic in 17% of the patients. Cutaneous symptoms predominated at onset (77%), followed by articular symptoms (30%). The p.Thr348Met and p.Arg260Trp NLRP3 mutations were significantly associated with the presence and absence of articular symptoms at onset, respectively. During followup, 86% of the patients had musculoskeletal symptoms, 88% had arthralgia, and 58% had arthritis, but only 9% had joint destruction. Tendinopathies occurred in 21.5% of the patients, tender points in 16.5%, and myalgia in 33%. Only 3 patients had typical knee deformities. Radiographs were rarely obtained. Except for bone deformities, osteoarticular symptoms occurred at similar frequencies in the different CAPS phenotypes.

CONCLUSION:

Joint manifestations were frequent in all CAPS phenotypes. Bone deformities were rare. Musculoskeletal manifestations varied within given families but tended to worsen over time.

PMID:
26245507
DOI:
10.1002/art.39292
[Indexed for MEDLINE]
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