Weak evidence suggests that it may benefit patients with dysphagia associated with IBM (Asia–Pacific IVIg Advisory Board 2004). The inflammatory myopathies are a group of IBM is an idiopathic inflammatory disorder of Diagnostic criteria section has been revised Description and Diagnostic Criteria three discrete disorders of skeletal muscle: DM, muscle. It is the most common inflammatory and updated. SWG observed that IVIg in PM and IBM. myopathy in individuals older than 50 years. IBM continues to be controversial. Clinically IBM presents with slowly progressive Although initial trials looked promising, These disorders are acquired and have in weakness. It is more common in men than long term treatment does not appear common the occurrence of significant muscle women (3:1). Along with proximal muscle justified. However there is an exception weakness and the presence of an inflammatory weakness, distal muscles are commonly involved. with regards to treating dysphagia which response within the muscle. The disease has a predilection for certain affects up to 70% IBM patients. This may The diagnosis of DM, PM or IBM is usually made muscles, especially the quadriceps and long even apply with the use of subcutaneous finger flexors, with prominent atrophy of the IVIg. (Muscle & nerve 2013;48(5):839-9. by neurologists or rheumatologists, and relies quadriceps muscle. on the combination of careful clinical evaluation, an elevated creatine kinase level, electromyography and muscle biopsy. Diagnosis is Diagnosis made by a neurologist, Yes By which Neurologist, rheumatologist or Unchanged. required speciality clinical immunologist rheumatologist or immunologist Diagnosis must be No By which verified speciality Exclusion Criteria Expert consensus does not recommend IVIg to IBM with limb weakness without dysphagia This exclusion criterion has been reworded treat the limb weakness of IBM. affecting function for consistency with other exclusion criteria. Indications Patients with PM or DM with significant muscle Patients with IBM who have dysphagia limiting Indication reworded to require biopsy weakness unresponsive to corticosteroids and dietary intake confirmation and clearer definition of other immunosuppressive agents. degree of dysphagia. SWG confirmed that there is no evidence that can be used to verify rapidly Patients with IBM who have dysphagia affecting progressive IBM and noted that the criteria function.
Weak evidence suggests that it may benefit patients with dysphagia associated with IBM (Asia–Pacific IVIg Advisory Board 2004). Description and Diagnostic Criteria The inflammatory myopathies are a group of three discrete disorders of skeletal muscle: DM, PM and IBM. These disorders are acquired and have in common the occurrence of significant muscle weakness and the presence of an inflammatory response within the muscle. The diagnosis of DM, PM or IBM is usually made by neurologists or rheumatologists, and relies on the combination of careful clinical evaluation, an elevated creatine kinase level, electromyography and muscle biopsy. IBM is an idiopathic inflammatory disorder of muscle. It is the most common inflammatory myopathy in individuals older than 50 years. Clinically IBM presents with slowly progressive weakness. It is more common in men than women (3:1). Along with proximal muscle weakness, distal muscles are commonly involved. The disease has a predilection for certain muscles, especially the quadriceps and long finger flexors, with prominent atrophy of the quadriceps muscle. Diagnostic criteria section has been revised and updated. SWG observed that IVIg in IBM continues to be controversial. Although initial trials looked promising, long term treatment does not appear justified. However there is an exception with regards to treating dysphagia which affects up to 70% IBM patients. This may even apply with the use of subcutaneous IVIg. (Muscle & nerve 2013;48(5):839-9. Diagnosis is required Diagnosis made by a neurologist, rheumatologist or immunologist Yes By which speciality Neurologist, rheumatologist or clinical immunologist Unchanged. Diagnosis must be verified No By which speciality Exclusion Criteria Expert consensus does not recommend IVIg to treat the limb weakness of IBM. IBM with limb weakness without dysphagia affecting function This exclusion criterion has been reworded for consistency with other exclusion criteria. Indications Patients with PM or DM with significant muscle weakness unresponsive to corticosteroids and other immunosuppressive agents. Patients with IBM who have dysphagia affecting function. Patients with IBM who have dysphagia limiting dietary intake Indication reworded to require biopsy confirmation and clearer definition of degree of dysphagia. SWG confirmed that there is no evidence that can be used to verify rapidly progressive IBM and noted that the criteria
inflammatory-myopathies-inclusion-body-myositis-ibm.DOCX