The New Insight of Neonatal Autoimmune Diseases

The New Insight of Neonatal Autoimmune Diseases

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.

Neonatal Autoimmune Disease

1. Neonatal lupus
2. Neonatal anti-phospholipid syndrome
3. Behcet’s disease
4. Neonatal autoimmune thyroid disease
5. Neonatal polymyositis and dermatomyositis
6. Neonatal scleroderma
7. Neonatal type I diabetes mellitus.

The autoinflammatory syndromes are a completely different category, but are also included in discussion of neonatal autoimmune diseases. The autoinflammatory syndromes include which all share a common pathophysiologic mechanism.

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 lupus

In neonatal lupus, the heart seems to be particularly susceptible. Primary autoimmunity in newborns, with the exception of familial autoinflammatory diseases, is virtually non-existent. Neonatal lupus is the most common presentation of autoimmunity in the newborn. But the characteristics defining neonatal lupus are not well defined and the presentation of neonatal lupus differs from that of classical lupus.

Neonatal anti-phospholipid syndrome

Antiphospholipid antibodies and the syndrome associated with them have provided clinical, laboratory, and experimental challenges, and their specific pathogenesis in pregnancy-related complications remains an evolving story. Antiphospholipid syndrome (APS) involves arterial thrombosis, venous thrombosis, or recurrent pregnancy loss in association with the laboratory demonstration of the presence of antiphospholipid antibodies. The two most commonly associated antibodies are anticardiolipin antibodies and the lupus anticoagulant. APS can have a direct negative effect on the developing placenta in pregnancy, and maternal thrombosis may result from the additive thrombogenic combination of antiphospholipid antibodies and the pregnancy state. Pregnancy complications associated with APS include recurrent first-trimester miscarriage, second- or third-trimester fetal death, and preterm delivery.

Treatment of APS in pregnancy is based primarily on consensus and expert opinion.
Neonate born from a patient previously diagnosed as primary APS. A male, preterm born twin infant, whose mother had been diagnosed as primary APS, developed thrombocytopenia, livedo reticularis, pericardial effusion and thrombosis of the left subclavian and external jugular veins concomitantly with severe respiratory tract infection soon after his delivery, that culminated with his death two months after the birth, in spite of the large spectrum antibiotic therapy and all supportive measures.

Laboratory findings included high titers of IgM anticardiolipin antibodies and moderate titers of IgG isotype and negative antinuclear antibody, configuring a case of neonatal APS. Neonatal APS is a rare clinical condition, with only a few cases described in the literature. Its occurrence may depend on the passage of antibodies through the placenta or, as it seems to have occurred in the present case, by the production of de novo antibodies by the fetus.

The present case illustrates the necessity of a higher surveillance of the neonates born from mothers with primary APS or systemic lupus erythematosus (SLE) for the eventual development of such complication.

Behcet’s disease

Behçet disease (BD) is characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. Hippocrates may have described Behçet disease in the fifth century BC; however, the first description of the syndrome was attributed to the Turkish dermatologist Hulusi Behçet in 1924. In 1930, the Greek physician Adamantiades reported a patient with inflammatory arthritis, oral and genital ulcers, phlebitis, and iritis. Since then, the syndrome has been referred to as Behçet disease.

Behçet’s disease is a rare immune-mediated systemic vasculitis that often presents with mucous membrane ulceration and ocular involvements. Behçet’s disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. Behçet’s original article in German together with an English translation and a brief modern commentary are available on-line. As a systemic disease, it can also involve visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, and neurological systems. This syndrome can be fatal, due to ruptured vascular aneurysms, or severe neurological complications

Neonatal autoimmune thyroid disease
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. In neonatal lupus, the heart seems to be particularly susceptible. Primary autoimmunity in newborns, with the exception of familial autoinflammatory diseases, is virtually non-existent. 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. Neonatal lupus is the most common presentation of autoimmunity in the newborn. But the characteristics defining neonatal lupus are not well defined and the presentation of neonatal lupus differs from that of classical lupus. Other neonatal autoimmune diseases involving the interaction between maternal antibodies and fetal/neonatal antigens include neonatal anti-phospholipid syndrome, Behcet’s disease, neonatal autoimmune thyroid disease, neonatal polymyositis and dermatomyositis, neonatal scleroderma and neonatal type I diabetes mellitus. 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 autoinflammatory syndromes are a completely different category, but are also included in discussion of neonatal autoimmune diseases. The autoinflammatory syndromes include the cryopyrin associated periodic syndromes (CAPS) – familial cold autoinflammatory syndrome (FCAS), neonatal onset multisystem inflammatory disease (NOMID) and 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.”

Neonatal scleroderma

Neonatal scleroderma were analysed showed and showed that the morbidity rate was higher in cold area than that in warm area. Cold and infectionwere principal causes induing the disease. There was positive correlation between low tem-perature and the prognosis. Pneumorrhagia was the main cause of death.The prognosis ofneonatal scleroderma could be judged with blood gas analysis.

Scleroderma is both a rheumatic disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage. Scleroderma refers to a group of rare chronic autoimmune diseases in which the skin and connective tissues tighten and harden; it is a progressive disease. Scleroderma occurs when the body produces and accumulates too much collagen in tissues. Systemic scleroderma may involve all connective tissue containing organs; however, some represent the main target of the disease. These include the skin, gastrointestinal tract, heart, lungs, and kidneys.

Neonatal type I diabetes mellitus.

Neonatal diabetes mellitus (NDM) is a monogenic form of diabetes that occurs in the first 6 months of life. It is a rare disease, occurring in only one in 100,000 to 500,000 live births. Infants with NDM do not produce enough insulin, leading to an increase in blood glucose. NDM can be mistaken for the much more common type 1 diabetes, but type 1 diabetes usually occurs later than the first 6 months of life. In about half of those with NDM, the condition is lifelong and is called permanent neonatal diabetes mellitus (PNDM). In the rest of those with NDM, the condition is transient and disappears during infancy but can reappear later in life; this type of NDM is called transient neonatal diabetes mellitus (TNDM). Specific genes that can cause NDM have been identified.

Symptoms of NDM include thirst, frequent urination, and dehydration. NDM can be diagnosed by finding elevated levels of glucose in blood or urine. In severe cases, the deficiency of insulin may cause the body to produce an excess of acid, resulting in a potentially life-threatening condition called ketoacidosis. Most fetuses with NDM do not grow well in the womb and newborns are much smaller than those of the same gestational age, a condition called intrauterine growth restriction. After birth, some infants fail to gain weight and growth as rapidly as other infants of the same age and sex. Appropriate therapy improves and may normalize growth and development.

Other neonatal autoimmune diseases involving the interaction between maternal antibodies and fetal/neonatal antigens include neonatal anti-phospholipid syndrome, Behcet’s disease, neonatal autoimmune thyroid disease, neonatal polymyositis and dermatomyositis, neonatal scleroderma and neonatal type I diabetes mellitus. 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 autoinflammatory syndromes are a completely different category, but are also included in discussion of neonatal autoimmune diseases. The autoinflammatory syndromes include the cryopyrin associated periodic syndromes (CAPS) – familial cold autoinflammatory syndrome (FCAS), neonatal onset multisystem inflammatory disease (NOMID) and Muckle-Wells syndrome, which all share a common pathophysiologic mechanism.

References:

  • Chang C. Neonatal autoimmune diseases: A critical review. J Autoimmun. 2012 May;38(2-3):J223-38.
  • Soares Rolim AM, et al. Neonatal antiphospholipid syndrome. Lupus. 2006;15(5):301-3.
  • James F. Smith Jr, Maurice L. Druzin. Perinatal Implications of the Antiphospholipid Syndrome. NEOREVIEWS Vol. 8 No. 5 May 1, 2007
    pp. e206 -e213
  • 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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2 responses to “The New Insight of Neonatal Autoimmune Diseases

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