Significance of Sjögren's syndrome and anti-cN1A antibody in myositis patients.

OBJECTIVE: We recently recorded a high prevalence of inclusion body myositis (IBM) in patients with Sjögren's syndrome (SS). Whether myositis patients with SS differ from myositis patients without SS in terms of the characteristics of the myositis is currently unknown. Anti-cytosolic 5'-nucleotidase 1 A (cN1A) has recently been proposed as a biomarker for IBM but is also frequent in SS. Whether anti-cN1A is independently associated with IBM is still an open question. We aimed to assess the significance of SS and anti-cN1A in myositis patients. METHODS: Cumulative data on all myositis patients (EULAR/ACR 2017 criteria) screened for SS (ACR/EULAR 2016 criteria) in a single centre were analysed. Ninety-nine patients were included, covering the whole spectrum of EULAR/ACR 2017 myositis subgroups and with a median follow-up of 6 years (range 1.0-37.5). The 34 myositis patients with SS (myositis/SS+) were compared with the 65 myositis patients without SS (myositis/SS-). RESULTS: . IBM was present in 24% of the myositis/SS+ patients vs 6% of the myositis/SS- group (P = 0.020). None of the IBM patients responded to treatment, whether they had SS or not. Anti-cN1A was more frequent in myositis/SS+ patients (38% vs 6%, P = 0.0005), independently of the higher prevalence of IBM in this group (multivariate P value: 0.02). Anti-cN1A antibody specificity for IBM was 0.96 (95% CI: 0.87, 0.99) in the myositis/SS- group but dropped to 0.70 (95% CI: 0.48, 0.85) in the myositis/SS+ group. INTERPRETATION: In myositis patients, SS is associated with IBM and with anti-cN1A antibodies, independently of the IBM diagnosis. As a consequence, anti-cN1A has limited specificity for IBM in myositis patients with SS.

[Diagnostic criteria for Sjögren's syndrome]. / Critères diagnostiques du syndrome de Gougerot-Sjögren.

Sjödgrens syndrome is a common autoimmune disease with highly variable clinical manifestations, making diagnosis difficult and sometimes delayed. Its specific clinical presentation combines oral and ocular dryness, asthenia and joint involvement. However, visceral involvement and potentially severe complications, including lymphoma, are frequently observed. These can be indicative of the disease and should lead to consider Sjögren's syndrome as a diagnosis. Internationally agreed upon diagnostic criteria were developed to improve the ability to make a definitive diagnosis, in order to provide follow-up and appropriate treatment measures as early as possible. These criteria, which include clinical, laboratory and histological measures, are easy to use in the daily practice, and make it possible to rule out the extensive differential diagnosis of sicca syndrome.

Recommendations for using TNFα antagonists and French Clinical Practice Guidelines endorsed by the French National Authority for Health.

The use of TNFα antagonists must follow specific guidelines to ensure optimal effectiveness and safety. The French Society for Rheumatology (SFR) and Task Force on Inflammatory Joint Diseases (CRI), in partnership with several French learned societies, asked the French National Authority for Health (HAS) to develop and endorse good practice guidelines for the prescription and monitoring of TNFα antagonist therapy by physicians belonging to various specialties. These guidelines were developed, then, validated by two multidisciplinary panels of experts based on an exhaustive review of the recent literature and in compliance with the methodological rules set forth by the HAS. They pertain to the initial prescription of TNFα antagonists and to a variety of clinical situations that can arise during the follow-up of patients receiving TNFα antagonists (infections, malignancies, pregnancy, vaccination, paradoxical adverse events, surgery, use in older patients, and vasculitides).

Neurogenic muscle hypertrophy.

We report the case of a 48-year-old man who presented with left calf hypertrophy 6 years after an episode of left S1 sciatica related to a herniated disk. Magnetic resonance imaging disclosed muscle hypertrophy. Electromyography showed left S1 radiculopathy with abnormal spontaneous muscle activity. Neurogenic muscle hypertrophy is a rare phenomenon that is chiefly seen when denervation occurs slowly and gradually. The typical patient is a middle-aged man who has a history of S1 radiculopathy. The soleus muscle is the main site of involvement. The pathophysiology is unclear but may involve type I fiber hypertrophy in response to the complex repetitive discharges recorded by electromyography. The natural history of neurogenic muscle hypertrophy is incompletely understood.