Systemic Inflammation in Duchenne Muscular Dystrophy: Association with Muscle Function and Nutritional Status

Abstract

Inflammation described in patients with Duchenne muscular dystrophy (DMD) may be related to loss of muscle function or to obesity. It is unknown if circulating proinflammatory cytokines (IL-6, IL-1, and TNF-α) levels are associated with muscle function. The purpose was to evaluate whether an association exists between systemic inflammation with muscle function and nutritional status in DMD patients. In 66 DMD patients without corticosteroid treatment, the following were evaluated in serum: cytokines (IL-1, IL-6, and TNF-α), C-reactive protein (CRP), leptin, adiponectin, and creatine kinase (CK). Muscle function was evaluated using Vignos Scale. Patients with better muscle function had the highest concentration of CK, IL-1, and TNF-α compared with less muscle function. No differences in IL-6 and adiponectin concentration were identified among groups with different levels of muscle function. Also, no differences were observed in the concentration of cytokines among groups with different nutritional status levels (underweight, normal weight, and overweight/obese). However, CRP and leptin were increased in the obese group compared with normal and underweight subjects. Systemic inflammation is increased in patients with better muscle function and decreases in DMD patients with poorer muscle function; nevertheless, systemic inflammation is similar among different levels of nutritional status in DMD patients.

Recovery of respiratory function in mdx mice co‐treated with neutralizing interleukin‐6 receptor antibodies and urocortin‐2

“…; Cruz‐Guzmán et al . ; Pelosi et al . ) and is of particular relevance due to its role as a myokine and muscle signalling molecule.…”

“…During the years of ambulation, plasma cytokine levels appear to be near maximal in DMD boys and begin to decline thereafter when patients become non‐ambulatory (Cruz‐Guzmán et al . ), perhaps due to the disuse of the lower limb musculature. IL‐6 is one of many cytokines reported to be elevated in DMD (Rufo et al .…”

Effects of non‐euphoric plant cannabinoids on muscle quality and performance of dystrophic mdx mice

“…Therefore, the lack of functional dystrophin inevitably compromises the structure of the sarcolemma membrane, thus causing progressive muscle damage and failure (Matsumura et al, ). The inflammatory response, activation of necrosis and exhaustion of regeneration cycles of muscle fibres are all known mechanisms leading to the exacerbation of the disease (Cruz‐Guzmán et al, ; Miyatake et al, ).…”

Muscle function and age are associated with loss of bone mineral density in Duchenne muscular dystrophy

“…In addition, chronic inflammation decreases muscular regeneration, aggravating the loss of muscle function, as we proposed in patients with DMD. It has been observed that in boys who have more advanced disease are nonambulatory, and inflammatory markers such as interleukin‐1 (IL‐1), TNF‐α, and C‐reactive protein (CRP) are lower compared with ambulatory boys with earlier‐onset disease . Loss of muscle function is associated with a reduction in bone density, because bone is controlled by muscle strength .…”

“…It has been observed that in boys who have more advanced disease are nonambulatory, and inflammatory markers such as interleukin-1 (IL-1), TNF-α, and C-reactive protein (CRP) are lower compared with ambulatory boys with earlier-onset disease. 11 Loss of muscle function is associated with a reduction in bone density, because bone is controlled by muscle strength. 10,12 Therefore, the loss of ambulatory function and muscle mass can lead to bone loss, as observed in children with DMD.…”