Sporadic-inclusion body myositis (s-IBM) is not so prevalent in Istanbul/Turkey: a muscle biopsy based survey

Abstract

In a muscle biopsy based study, only 9 out of 5450 biopsy samples, received from all parts of greater Istanbul area, had typical clinical and most suggestive light microscopic sporadic-inclusion body myositis (s-IBM) findings. Two other patients with and ten further patients without characteristic light microscopic findings had referring diagnosis of s-IBM. As the general and the age-adjusted populations of Istanbul in 2010 were 13.255.685 and 2.347.300 respectively, the calculated corresponding 'estimated prevalences' of most suggestive s-IBM in the Istanbul area were 0.679 X 10(-6) and 3.834 X 10(-6). Since Istanbul receives heavy migration from all regions of Turkey and ours is the only muscle pathology laboratory in Istanbul, projection of these figures to the Turkish population was considered to be reasonable and an estimate of the prevalence of s-IBM in Turkey was obtained. The calculated 'estimated prevalence' of s-IBM in Turkey is lower than the previously reported rates from other countries. The wide variation in the prevalence rates of s-IBM may reflect different genetic, immunogenetic or environmental factors in different populations.

Figure 1.

A) Atrophy of the long finger flexors and inability to flex the fingers in one of our patients with typical clinical findings of s-IBM. B) Inflammation and rimmed vacuoles in one of our patients with most suggestive s-IBM histopathology (Engel's modified trichrome (E-MGT) stain, 250X) C) Inflammation and rimmed vacuoles in higher magnification in one of our patients with most suggestive s-IBM histopathology (E-MGT stain, 500X) D) Sarcolemmal MHC-1 staining in most fibers in the same patient (500X).