2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies
and their major subgroups.
Levels of the alpha-chemokines CXCL9 and CXCL10, which are expressed in muscle affected by idiopathic inflammatory myopathies
such as dermatomyositis, declined strongly over the course of 12 weeks of treatment to an extent that was just shy of statistical significance.
Idiopathic inflammatory myopathies
(IIMs) are heterogeneous disorders characterized by chronic muscle weakness and muscle fatigue and mononuclear cell infiltration into skeletal muscle. The most common subgroups in adults are dermatomyositis (DM), polymyositis (PM), amyopathic DM (ADM), inclusion body myositis (IBM), and juvenile DM (JDM).
Net proceeds may be used by the company to advance KZR-616 for the treatment of lupus and lupus nephritis, to advance KZR-616 for the treatment of idiopathic inflammatory myopathies
and up to three additional autoimmune indications, to advance discovery and preclinical development in its protein secretion programme, to fund other research and development activities, working capital as well as other general corporate purposes.
A relation was suggested between IL-1Ra allele 2 and severe forms of Sjogren's syndrome, skin disease in systemic lupus erythematosus, juvenile idiopathic inflammatory myopathies
, and juvenile chronic arthritis, especially in patients with a prolonged oligoarticular involvement (17, 22-24).
Anti-ARS antibodies are known as myositis-specific autoantibodies and have been extensively detected in idiopathic inflammatory myopathies
(15) and in patients with inflammatory myopathies overlapping with RA.
Guzman, "Idiopathic inflammatory myopathies
: clinical approach and management," Frontiers in Neurology, vol.
Etiologies of NB Myopathy NB in other neuromuscular disorders AD: NEB, ACTA1, TPM3, TPM2 Myopathy Idiopathic inflammatory myopathies
Acute alcoholic myopathy AR: ACTA1, TPM3, TPM2, TNNT1, Myotonic dystrophy CFL2, KBTBD13, KLHL40, Sarcoglycanopathies KLHL41, LMOD3, MYPN, MYO18B Mitochondrial myopathy GYG1 polyglucosan body myopathy Late-onset Pompe disease Acquired Neuropathy MGUS Spinal muscular atrophy HIV-associated myopathy Amyotrophic lateral sclerosis Charcot-Marie-Tooth disease Other Hypothyroidism Chronic renal failure Keys: genes are written in italic font; AD, autosomal dominant; AR, autosomal recessive; NB, nemaline body; MGUS, monoclonal gammopathy of undetermined significance.
The juvenile idiopathic inflammatory myopathies
(JIIMs) are heterogeneous, systemic, autoimmune diseases with onset in childhood; they are characterized by weakness, chronic inflammation of the skeletal muscles, and typical skin rashes.
A systematic review of adult onset clinically amyopathic dermatomyositis (Dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies
. J Am Acad Dermatol.
In the idiopathic inflammatory myopathies
(IIM), do reactive oxygen species (ROS) contribute to muscle weakness?
The GI manifestations of the idiopathic inflammatory myopathies
include uncoordinated swallowing, uncoordinated esophageal peristalsis, and hiatal hernia with reflux and stricture formation .