Neonatal autoimmune diseases are distinctly rare. Most neonatal autoimmune diseases result from the transplacental transfer of maternal antibodies directed against fetal or neonatal antigens in various tissues.
While autoantibodies have been detected in patients with neonatal autoimmune disease, the pathogenic role of autoantibodies has not been well defined. Other mechanisms may play a role in the development of neonatal autoimmunity, including fetal/maternal microchimerism and aberrant apoptosis of fetal cells.
The pathophysiologic basis for the development of neonatal autoimmunity is not entirely clear, but differences in the neonatal immune system compared with the adult immune system, as well as unique characteristics of target antigens in the newborn period may be important factors.
The autoinflammatory syndromes are a completely different category, but are also included in discussiony of neonatal autoimmune diseases. The autoinflammatory syndromes include which all share a common pathophysiologic mechanism.
Neonatal autoimmune diseases involving the interaction between maternal antibodies and fetal/neonatal antigens include neonatal lupus, neonatal anti-phospholipid syndrome, Behcet’s disease, neonatal autoimmune thyroid disease, neonatal polymyositis and dermatomyositis, neonatal scleroderma and neonatal type I diabetes mellitus.
The autoinflammatory syndromes include :
1. The cryopyrin associated periodic syndromes (CAPS)
2, Familial cold autoinflammatory syndrome (FCAS)
3. Neonatal onset multisystem inflammatory disease (NOMID)
4. Muckle-Wells syndrome, which all share a common pathophysiologic mechanism.
Neonatal polymyositis and dermatomyositis
Dermatomyositis (DM) is a connective-tissue disease related to polymyositis (PM) and Bramaticosis that is characterized by inflammation of the muscles and the skin. The cause is unknown, but it may result from either a viral infection or an autoimmune reaction. In the latter case it is a systemic autoimmune disease. Many people diagnosed with dermatomyositis were previously diagnosed with infectious mononucleosis and Epstein-Barr virus. Some cases of dermatomyositis actually “overlap” are combined with other autoimmune diseases such as: Sjögren’s syndrome, lupus, scleroderma, or vasculitis. Because of the link between dermatomyositis and autoimmune disease, doctors and patients suspecting dermatomyositis may find it helpful to run an ANA – antinuclear antibody – test, which in cases of a lupus-like nature may be positive (usually from 1:160 to 1:640, with normal ranges at 1:40 and below). Several cases of polymyositis and dermatomyositis were reported as being triggered by the use of various statin drugs used to control blood cholesterol. Muscle biopsies of these patients showed rhabdomyolysis, and degeneration and regeneration of muscle tissue.
High blood levels of creatine kinase (CPK) showed greater than 5 times the normal levels also supporting the rhabdomyolysis findings[clarification needed]. CPK is an enzyme found mainly in the heart, brain, and skeletal muscle that tends to rise when inflammation occurs during normal exercising. When these levels rise in excess of the normal level (less than 200), and become more than five times higher, severe cell damage to the muscles, brain, and heart ensue[clarification needed]. The higher the CPK, the greater the cellular damage done. Extremely high levels of CPK cause rhabdomyolysis to these muscles and organs[clarification needed]. Without treatment, kidney damage occurs and death in the more severe cases. Confirmed polymyositis with the skin signs is known as dermatomyositis which amplifies the problem to various cancers.
Some cases of dermatomyositis are a paraneoplastic phenomenon, indicating the presence of cancer. In cases involving cancer, the cancer is usually pre-existent, with removal of the cancer resulting in remission of the dermatomyositis. The onset of a rash in patients with pre-existing myositis requires investigation of the neoplastic possibility.
In his 1988 article, Clinical pathologic correlations of Lyme disease by stage, noted Lyme disease researcher Dr. Alan Steere observed, ” the perivascular lymphoid infiltrate in clinical myositis does not differ from that seen in polymyositis or dermatomyositis. All of these histologic derangements suggest immunologic damage in response to persistence of the spirochete, however few in number.”
1. Chang C. Neonatal autoimmune diseases: A critical review. J Autoimmun. 2012 May;38(2-3):J223-38.
2. Soares Rolim AM, et al. Neonatal antiphospholipid syndrome. Lupus. 2006;15(5):301-3.
3. James F. Smith Jr, Maurice L. Druzin. Perinatal Implications of the Antiphospholipid Syndrome. NEOREVIEWS Vol. 8 No. 5 May 1, 2007
pp. e206 -e213
4. Monogenic Forms of Diabetes: Neonatal Diabetes Mellitus and Maturity-onset Diabetes of the Young at National Diabetes Information Clearinghouse, a service of the National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health. NIH Publication No. 07–6141. March 2007.
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