Inclusion body myositis associated with Sjögren’s syndrome

Abstract

Inclusion body myositis (IBM) belongs to the group of idiopathic inflammatory myopathies. It is a poorly understood disease, which affects skeletal muscles. IBM usually occurs as an isolated condition, but in some cases, it may be associated with another autoimmune disorder, Sjögren’s syndrome. We report a case of a 47-year-old woman with headaches, symptoms of trigeminal neuralgia, progressive weakness in muscles of the upper and lower extremities and symptoms of dry eyes and mouth. On admission, creatine kinase level was increased to 6,956 IU/mL and lactate dehydrogenase (LDH) to 1,011 U/L in the serum. The increase in inflammatory factor (CRP, ESR) levels was not found. The diagnosis of inclusion body myositis associated with Sjögren’s syndrome was established on the basis of clinical picture and diagnostic tests. In this therapy, methotrexate and methylprednisolone were administered. The considerable improved muscle strength in the upper and lower extremities, improved speech and swallowing, disappearance of headache and reduction in CPK and LDH levels were found 8 months after establishing the diagnosis. Treatment with methotrexate and methylprednisolone improved the clinical symptoms and quality of life of this patient and may offer a therapeutic option for some patients with IBM and concomitant Sjögren’s syndrome.

Inclusion‐body myositis and primary Sjögren syndrome: mechanisms for shared etiologies

“…Although inflammatory myopathy is rare in pSS, there are occasional reports of sIBM in patients with pSS . One study described 8 of 36 (22%) of patients with pSS and myalgia, who underwent muscle biopsy, showed histopathological features of IBM, although none had clinical features characteristic of this condition .…”

Corticoid Therapy for Overlapping Syndromes in an HIV-positive Patient

“…Immunohistochemical analyses of the muscle tissue in cases of HIV-associated PM tend to show a significant decrease in endomysial CD4 + T lymphocytes, although these findings cannot be used to distinguish the condition from cases of non-HIV PM (17). Therefore, the muscle infiltrates observed in patients with SS consist of a mixture of B and T cells, with the proportions of CD4 + and CD8 + lymphocytes varying based on the individual case (18). In this manner, the molecular mechanisms underlying the development of myositis associated with SS and/or PM may have contributed to the onset of inflammatory myopathy noted in the present patient.…”

“…The generation of autoantibodies in patients suffering from HIV infection suggests polyclonal B cell function hyperactivity (18). In fact, light chain restriction without IgH rearrangement has been detected in reactive lymph nodes in patients with Castleman's disease, as well as autoimmune and viral disorders, such as herpes virus 6, Epstein-Barr virus (EBV) and HIV (32).…”

The Clinical and Pathological Spectrum of Idiopathic Inflammatory Myopathies : Implications for pathogenesis, classification and diagnosis

“…Several patients with Sjögren syndrome have also been diagnosed with IBM [126], and in some of these cases, the muscle weakness was reported to respond to therapy [127,128]. It has further been suggested that IBM patients with…”

“…Sjögren syndrome may represent a subset of IBM [11,128], that may improve when treated early [127]. In a study of patients with rheumatoid arthritis, a sub-group of patients with myositis, either had a raise of erythrocyte sedimentation rate, not accounted for by synovitis, or of creatinine phosphate (CK), where muscle tissue of these patients was reported to exhibit de novo synthesis of rheumatoid factor and IgM [129], which was interpreted as evidence of a pathogenic overlap in these diseases.…”