Factors affecting outcome in myasthenia gravis.

INTRODUCTION AND METHODS: Information from myasthenia gravis (MG) patients treated and evaluated for at least 2 years between 1980 and 2014 was reviewed to assess the effect of demographics, antibody status and titer, thymus histology, and clinical severity on outcome after 2, 5, and 10 years of treatment. RESULTS: Among 268 patients, 74% had acetylcholine receptor antibodies, 5% had muscle specific tyrosine kinase-antibodies, and 22% had neither. Optimal outcome was achieved by 64% of patients at 2 years of follow-up, 73% at 5 years, and 75% after 10 years. Optimal outcome was achieved more often in patients with late onset, in those who had thymectomy, and in those with ocular-only disease at maximum severity. The only consistent independent predictor of optimal outcome was onset after age 50 years on multivariate analysis. CONCLUSIONS: Prognosis is favorable for the majority of MG patients, regardless of age, maximum disease severity, or antibody status. Muscle Nerve, 2016 Muscle Nerve 54: 1041-1049, 2016.

Incidence Trends and Mortality of Giant Cell Arteritis in Southern Norway.

OBJECTIVE: Southern Norway consists of a homogeneous population of nearly 300,000 inhabitants and is an ideal epidemiologic setting. We aimed to explore potential changes in incidence of giant cell arteritis (GCA) in Southern Norway from 2000-2013, with comparisons of previous reports from the same population cohort from 1987-1994 and 1992-1996, and to investigate the mortality rates of GCA over a period of 14 years. METHODS: All patients diagnosed with GCA during January 1, 2000 to December 31, 2013 were identified through the electronic health records and biopsy findings databases at our clinic. The diagnosis of GCA and information about death was confirmed by reviewing the patients' hospital records. Inclusion criteria were: 1) fulfillment of the American College of Rheumatology 1990 criteria for GCA, or 2) histologically proven GCA, or 3) confirmed arteritis of the large or medium-sized vessels by imaging. RESULTS: A total of 206 patients were included, and 147 (72%) were females. The annual incidence rate of GCA per 100,000 inhabitants age ≥50 years was 16.8 (95% confidence interval [95% CI] 14.6-19.2), 24.5 for females (95% CI 19.2-26.5), and 10.2 for males (95% CI 7.9-13.2). Forty-six patients (22%) died (24 women, 22 men). The overall standardized mortality ratio was 1.05 (95% CI 0.77-1.38), 0.92 for females (95% CI 0.61-1.35), and 1.38 for males (95% CI 0.88-2.05). Overall survival rate was significantly higher in females compared to males (P < 0.001). CONCLUSION: GCA incidence is not increasing. We did not find excess mortality; however, males seem to have a worse survival rate compared to females.

Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL).

BACKGROUND: Common variable immunodeficiency (CVID) and other primary antibody deficiencies (PAD) are a heterogeneous group of > 300 congenital disorders affecting the immune system. Until recently, efforts to measure health-related quality of life (QoL) in PAD patients have utilised generic QoL tools and disease-specific tools for other conditions. Still, the full impact of the disease is probably not understood. We evaluated the performance of the CVID_QoL, a novel disease-specific QoL instrument for adults with CVID, on Norwegian PAD patients and compared the results to those of the generic WHOQOL-BREF. METHODS: Respondents were recruited through the Norwegian Centre for Rare Disorders' patient database. Included patients fulfilled the following criteria (all three): 1.) Age ≥18 years, 2.) a PAD diagnosis, 3.) currently on immunoglobulin therapy. The CVID_QoL is a 32-item questionnaire. Global CVID_QoL scores were compared between Norwegian PAD patients and Italian CVID patients. RESULTS: In total, 83 PAD patients filled out the CVID_QoL, 63% had CVID, 76% were females. 32 patients filled out the WHOQOL-BREF. Feasibility was high (<1% missing). Internal consistency for the emotional- (Cronbach's α-value = 0.91) and relational functioning (α =  0.77) subscales was high, but questionable for the gastrointestinal and skin symptoms subscale (α =  0.66). Convergent validity varied from weak to strong (range 0.3-0.8). Floor and ceiling effects were present. CONCLUSIONS: Although many disease-specific characteristics are probably shared with CVID and other PAD, the CVID_QoL captures some, but not all, dimensions of PAD patients' QoL. More evaluations of the CVID_QoL's performance in different contexts are needed.