Seasonal effect on fatigue, pain and dryness in primary Sjögren's syndrome.

Background: To assess the presence of a seasonal effect on fatigue, pain and dryness in primary Sjögren's syndrome (pSS).

Methods: Data (date; visual analogue scales (VAS) for pain, fatigue and dryness) were extracted from three randomised placebo-controlled trials (RCTs) evaluating infliximab (TRIPSS; n = 103 patients), hydroxychloroquine (JOQUER; n = 120 patients) and rituximab (TEARS; n = 120 patients) and from the 5-year follow-up of the ASSESS prospective cohort (n = 395 patients). Data were analysed at each visit for each patient, according to the day, the month of the year and the season.

Usefulness of F-Labeled Fluorodeoxyglucose-Positron Emission Tomography for the Diagnosis of Lymphoma in Primary Sjögren's Syndrome.

Objective: The usefulness of positron emission tomography-computed tomography (PET-CT) with F-labeled fluorodeoxyglucose ( F-FDG) for the diagnosis of lymphoma in patients with primary Sjögren's syndrome (SS) is unclear, since the abnormalities it reveals may be due to systemic manifestations of SS. This study was undertaken to compare F-FDG-PET-CT in patients with primary SS with lymphoma and those without lymphoma in order to identify patterns associated with lymphoma.

Methods: A retrospective study was conducted in 2 centers and included patients who met the American College of Rheumatology/European League Against Rheumatism 2016 criteria for primary SS and had undergone PET-CT.

[Pregnancy and antiphospholipid syndrome].

Antiphospholipid syndrome (APS) is associated with a risk of obstetrical complications, affecting both the mother and the fetus. Obstetrical APS is defined by a history of three consecutive spontaneous miscarriages before 10 weeks of gestation (WG), an intra-uterine fetal death after 10 WG, or a premature birth before 34 WG because of severe pre-eclampsia, eclampsia or placental adverse outcomes (intrauterine growth retardation, oligohydramnios). Pregnancy in women with a diagnosis of obstetric APS is at increased risk for placental abruption, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome and thrombosis that may be part of a catastrophic antiphospholipid syndrome (CAPS).

Myopathies related to systemic sclerosis: a case-control study of associated clinical and immunological features.

Objective: Little is known about systemic sclerosis (SSc)-related myopathy. We aimed to compare the clinical and immunological features of SSc patients with or without associated myopathy.

Methods: Forty SSc patients with myopathy, defined by myalgia or muscle weakness associated with creatine kinase (CK) more than five times the upper limit range or myopathic electromyography (EMG) or abnormal myopathology, were identified from the records of four French hospital centres.

A descriptive and prognostic study of systemic sclerosis-associated myopathies.

Objectives: To describe the clinical characteristics and muscle pathological features of patients with systemic sclerosis (SSc) and myopathy and analyse their impact on muscle outcome.

Methods: Thirty-five patients with myopathy and available muscle biopsy were restrospectively investigated from the charts of four hospital centres.

Results: Twenty-six (74%) cases had diffuse SSc.

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature.

Background: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy.

Assessing disability and quality of life in systemic sclerosis: construct validities of the Cochin Hand Function Scale, Health Assessment Questionnaire (HAQ), Systemic Sclerosis HAQ, and Medical Outcomes Study 36-Item Short Form Health Survey.

Objective: To assess the construct validity of the Cochin Hand Function Scale (CHFS) and the relevance of using aggregate scores for the scleroderma Health Assessment Questionnaire (sHAQ) and Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) in systemic sclerosis (SSc).

Methods: We evaluated 50 patients with SSc (mean +/- SD age and disease duration 54 +/- 12 years and 9 +/- 8 years, respectively), of which 26 had limited cutaneous SSc (lcSSc) and 23 diffuse SSc (dSSc). Quality of life was assessed by the SF-36, global disability by the Health Assessment Questionnaire (HAQ) and sHAQ, and hand disability by the CHFS.

Rituximab alone or in association with corticosteroids in the treatment of acquired factor VIII inhibitors: report of two cases.

Acquired haemophilia is a factor VIII (FVIII) deficiency due to autoantibodies directed against FVIII that can be responsible for severe haemorrhage. The therapeutic approach, in addition to the treatment of bleeding episodes with clotting factor infusion, relies on corticosteroids (CS), cyclophosphamide (CYC), and/or high-dose intravenous immunoglobulins (IVIg). However, the efficacy of IVIg is limited, and CS and CYC may cause a number of adverse effects.