Epidemiology, Survival, and Clinical Characteristics of Inclusion Body Myositis

Abstract

Objective: We performed a population-based study on inclusion body myositis with the primary aims to define the prevalence, survival rate, and incidence, and to investigate the symptom profiles associated with disease duration and sex over a 33-year period. Methods: Patients diagnosed between 1985 and 2017 in Region Västra Götaland, Sweden, were identified according to the European Neuromuscular Centre diagnostic criteria from 2011. Results: We identified 128 patients, 89 men and 39 women, with the strict clinicopathological definition of inclusion body myositis. The prevalence was 32 per million inhabitants, 19 per million women and 45 per million men, by December 31, 2017. Mean incidence was 2.5 per million inhabitants and year. Mean age at symptom onset was 64.4 years with quadriceps weakness being the most common presenting symptom followed by finger flexor weakness. Dysphagia was a common presenting symptom being more frequent in women (23%) than men (10%) and was during the disease course reported in 74% of men and 84% of women. Seventy-three patients were deceased, with a mean survival of 14 years from symptom onset. Survival rates from both diagnosis date and symptom onset were decreased compared to the matched population. Twenty-one percent of the patients had an additional autoimmune disease. A cross-sectional analysis of autoantibodies in 50 patients and 28 matched controls showed autoantibodies to cytosolic 5 0 -nucleotidase 1A in 40% of the patients and 3.6% of controls. Interpretation: Inclusion body myositis is an autoimmune disease with decreased survival rate and with marked sex differences in both prevalence and clinical manifestations.

Cost of illness in inclusion body myositis: results from a cross-sectional study in Germany

“…Of the 41.5% utilizing IVIg treatment in the recall period, the treatment duration ranged from 0.4 to 10 years. A study from Sweden observed that 82% of the IBM patients ever tried immunomodulating treatment [ 61 ]. Nevertheless, there is yet no international consensus on the effectiveness of IVIg treatment in IBM, although positive treatment responses are observed [ 13 , 62 ].…”

Pulmonale Beteiligung bei idiopathischen inflammatorischen Myopathien

“…Ein Ansprechen auf eine immunsuppressive Therapie besteht nicht, sodass eine autoimmune Ursache kontrovers diskutiert wird. Jedoch besteht bei bis zu 15 % der Patienten eine begleitende Autoimmunerkrankung und ein Nachweis von anti-cytosolische-5Nucleotidase-Antikörper (Anti-cN1A) [51]. Eine pulmonale Beteiligung im Sinne einer ILD ist untypisch.…”

Inclusion body myositis: Update on the diagnostic and therapeutic landscape

“…Most epidemiologic studies in IBM focused on estimating the incidence and prevalence of the disease. IBM affects males about twice as common as females, with a prevalence varying from 1 to 182 per million among those aged 50 and older (4)(5)(6)(7)(8). This variability in the reported prevalence is at least in part due to variability in case ascertainment methods and the used diagnostic criteria.…”

“…Furthermore, dysphagia is highly prevalent in IBM, with aspiration pneumonia, in addition to respiratory complications of the disease, being the most common cause of death ( 9 , 14 – 16 ). As a result, IBM is associated with a modest decrease in longevity, with a 10-year survival of 36–42% compared to 59% in population controls, and a mean age at death of 79.3 years compared to 83.6 in controls ( 8 – 10 ).…”