IgG4-related disease (IgG4-RD) is a newly recognized, multiorgan, fibro-inflammatory disease characterized by mass forming lesion with dense lymphoplasmacytic infiltration with large numbers of IgG4+ plasma cells, storiform fibrosis and sometimes obliterative phlebitis. IgG4-RD was first described in 2001 by Japanese researchers in patients with autoimmune pancreatitis with high levels of serum IgG.[1, 2]
IgG4-RD usually affects individuals of middle to old age, with an onset at 50–70 years, although few paediatric cases have been described. Males are more commonly affected especially for IgG4-related pancreatitis with an M:F ratio of 3:7. However, IgG4-related dacryoadenitis and sialadenitis may occur more frequently among
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Enlargement of one or more of trigeminal nerve branches is another feature of IgG4-ROD. The most commonly involved branches are the infraorbital nerve (ION) and the frontal nerve. Other nerves may be involved such as perioptic nerves, paravertebral nerves and great auricular nerve. The disease involves mainly the epineurium and the affected nerves are infilterated by masses of lymphocytes, plasma cells, eosinophils and large numbers of IgG+ plasma cells.[20-23]
EOM enlargement is not rare in IgG4-ROD especially in cases with enlarged orbital nerves. Single or multiple muscles may be involved during the disease course in the following order of frequency: inferior rectus, followed by superior rectus-levator complex, lateral rectus, medial rectus, inferior oblique and superior oblique. Histopathologically, the muscle biopsy shows a mixed and dense infiltration with polyclonal B- and T-cells with some fibrosis.[24, 25]
IgG4-ROD may also involve the lacrimal drainage apparatus.[26] Furthermore, sclera and conjunctival affection and nongranulomatous anterior uveitis have also been reported. [27, 28]
There is a correlation between IgG4-RD and ocular adnexal xanthogranulomatous disease in which a high
Normocephalic atraumatic. Pupils equally round and reactive to light, extraocular motions intact. Oral cavity shows oropharynx clear but slightly dried mucosal membranes. TM (tympanic membranes) clear. Neck, supple. There is no thyromegaly, no JVD. No cervical supraclavicular, axillary, or inguinal lymphadenopathy.
• A family history of another autoimmune disease, including type 1 diabetes and rheumatoid arthritis.
In the eyelid the rash of shingles can cause an inflammation of the eyelids that comes to block the vision. Lashes may fall, and scarring may occur due to rupture of the blisters. In the cornea the virus invades, it can cause ulcers and inflammation that will lead to a permanent cicatrization. This can result in blurred vision and blindness. You may also suffer from a loss of sensation in the cornea; For example, you will not be able to tell if you have a flap or if you have grit in your eye. On the uvea, including the iris. Inflammation of the iris occurs frequently when you suffer from shingles in the eye and this can lead to permanent scarring. It can also cause fluid pressure inside the eye to increase, increasing the chances of developing glaucoma. In the case of retinal infection, the cells to begin dying, creating blurred vision and pain in the eye. If left untreated, infection can lead to permanent blindness. A retinal detachment may also occur. If shingles invades the optic nerve it will inflame it, which can cause nerve damage similar to those caused by glaucoma, resulting in loss of
This disease affects the nerve cells by enabling them to send electric impulses to the muscles which ultimately makes the muscle wither away and stop working. The nerves targeted are actually
Myasthenia Gravis affects the neuromuscular junction. Neuromuscular junction is the connection or meeting place of the nerve and a muscle fiber. The nerves communicate with the muscle fiber by telling it to move or contract. The nerves
Myasthenia gravis [MG] is a rare, autoimmune neuromuscular disorder. The incidence rates has been reported as 2-7/10000 in central and western Virginia ( Thanvi ,2004).The onset of [MG] is depends on age and gender .In patients younger than 40 years, women are more susceptible than man to [MG],with a ratio of 7:3 :Above the age 50 [MG] are slightly more common in men with a ratio 3:2.Myasthenia gravis are very rare in children.Juvinile [MG] is an autoimmune disorder ,while congenital [MG] results from genetic mutations that impair neuromuscular transmission. It has been suggested that incidence of [MG] falls after 70 years of age. A recent study using AChR antibody as a diagnostic
SLE is a systemic autoimmune disease, where the body’s immune system wrongly attacks healthy tissue, and affects many of the internal organs in the body. SLE is most likely to damage the heart, skin, lungs, kidneys, joints, blood vessels, liver, and nervous system. SLE is a chronic inflammatory disease understood to be type III hypersensitivity responses, with some type II hypersensitivity involvement. Jillian’s complete blood count revealed she has low red blood cell and hemoglobin levels. Her result was 2.8 cells/mcL while the reference range is 3.90-5.03 cells/mcL for red blood cell count and 10.0 g/dL while the reference range is 12.0-15.5 g/dL for hemoglobin, resulting in anemia. With Type II hypersensitivity and hemolytic anemia, IgM antibodies bind to red blood cells and causes the agglutination of the red blood cells, resulting in a low red blood cell count. Hemolytic anemia explains Jillian’s symptoms of fatigue and shortness of breath. An indirect consequence of hemolytic anemia is the splenomegaly and pre-hepatic jaundice. The red blood cell breakdown and excretion of bilirubin resulted in Jillian’s high bilirubin total count in the blood chem panel, her value was 2.1 MG/DL when the average reference range is 0.2-1.3 MG/DL. With type III hypersensitivity immune
Guillain-Barre Syndrome (GBS) is a rare autoimmune disease. This is where an individual’s own immune system attacks and destroy healthy body tissue. The exact cause of this syndrome is unknown. However, once triggered the immune system begins to attack the myelin sheath in the brain, particularly, your peripheral nervous system (PNS). The PNS connects the brain and spinal cord (central nervous system – CNS) to the rest of the body. The myelin sheath main function is to ensure fast propagation of nerve impulses. When damaged it can often result in muscle weakness or paralysis.
To understand autoimmune diseases, the general characteristic of autoimmunity must be addressed. Autoimmunity is defined, in short, as “ misdirected immune response”. A healthy person is equipped with the mechanisms necessary to defend the body from pathogens within the immune system. When autoimmunity is present in an individual, something within the immune system is
The signs fall into two categories: optic neuritis and transverse myelitis. Optic neuritis is inflammation of the optic nerve and can result with pain inside the eye, unclear vision, or blindness. Optic neuritis usually occurs in one eye, but can happen in both. Transverse myelitis is inflammation of the spinal cord. This inflammation can result in pain, weakness, numbness, or paralysis of the arms and legs. It could also result in loss of control of the bladder and bowel. Side effects could result in nausea, hiccups, headaches, or a stiff neck. Symptoms and signs for children who have neuromyelitis optica include confusion, seizures, or
Umehara F, Matsumuro K, Kurono Y, Arimura K, Osame M, Kanzaki T. Neurologic manifestations of Kanzaki disease. Neurology. 2004 May 11;62(9):1604-6. PubMed citation
Hyper-IgE syndrome occurs because of the mutations in STAT3 gene where this gene is important to provide instructions in making proteins. The mutations were either missense
With autoimmune diseases, the underlying issue is similar. The body's immune system becomes unable to recognize healthy cells from diseased cells. Consequently, the immune’s own defense mechanism produces antibodies that attack the normal tissues and cells. Autoimmune diseases
The case-control study was performed in Denmark in the Copenhagen and Aarhus university hospitals. The sample was taken from the population of patients that were attending certain departments in the child psychiatry units. This register study compared the rates and types of autoimmune disease in the parents (Mourdisen et al., 2007). There were approximately 111 patients, along with their parents, used; 82 males and 29
There was a statistically negative correlation between n-Tregs percent and IL10b&IL10LPS, p<0.05. There was a statistically positive correlation between IL10b&IL10LPS and RC, p<0.001, while there was a statistically negative correlation with Hp, p<0.01 and p<0.05 respectively. Conclusion: Although there may be multiple factors that contribute to the induction of warm AIHA, our data indicate that defective suppressive activity of n-Treg cells and IL10/IL12 imbalance could play an essential role in the onset and/or maintenance of this autoimmune disease. Further investigations in 1ry AIHA syndrome in which autoimmunity is a significant feature, holds the promise of identifying new molecular defects that will further elucidate basic mechanisms of self tolerance and provide new targets for therapy.