orbital amyloidosis
Introduction
Introduction to orbital amyloidosis Orbital amyloidosis can occur in the orbital lacrimal gland, in the orbital and conjunctival tissues, is a lesion caused by amyloid deposition, clinically rare, lesions can cause eyeball protrusion, ptosis, eye movement disorder Eyelid bleeding (spontaneous) and decreased vision. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: eye movement disorders
Cause
Causes of orbital amyloidosis
Cause:
The reason is unknown. This lesion is a kind of lesion involving plasma cells, which may be related to immune dysfunction. Some cases are secondary to long-term ocular infection and chronic inflammation, vasculitis, cavernous vascular disease, etc. In myeloma (multiple), macroglobulinemia, B cell malignant lymphoma and the like.
(two) pathogenesis
The pathogenesis is unknown. Whether it is secondary or primary amyloidosis, plasma cells are involved in the pathological process, so it may be related to the body's immune dysfunction. Some cases have a certain family tendency, indicating genetics, protein immunoelectrophoresis. The high peak of IgM in the brain suggests macroglobulinemia.
Prevention
Orbital amyloidosis prevention
Eyelid amyloidosis prevention:
1. Because the cause of amyloidosis is unclear, there is no way to prevent primary amyloidosis.
2. Secondary amyloidosis can only initiate inflammatory diseases of amyloidosis, such as tuberculosis and rheumatoid arthritis, by prophylactic or effective treatment. If drugs can be used to control rheumatoid arthritis, the likelihood of developing secondary amyloidosis is reduced.
Complication
Orbital amyloidosis complications Complications, eye movement disorders
Often complicated by eye muscle hypertrophy, eye movement disorders, etc., because amyloid lesions are not limited to local, can be multiple systems and multiple organs, so the following complications may be combined:
1. Combined with renal diabetes insipidus, hyperkalemia, and renal failure.
2. Clinically, congestive heart failure is often associated with progressive and intractable episodes. Heart failure caused by amyloidosis is difficult to treat, and individual patients are extremely sensitive to digitalis, so that severe or even fatal arrhythmias occur. If the conduction system is involved, it can cause conduction block, atrial fibrillation, atrial flutter and ventricular arrhythmia. This is often a late manifestation of primary amyloidosis with a very poor prognosis.
3. Combined portal hypertension and esophageal variceal bleeding and spontaneous liver rupture. In addition, amyloid deposits can also be present in the gallbladder and pancreas.
4. Combined with airway obstruction, dyspnea, atelectasis, pleural effusion, secondary infection.
Symptom
Symptoms of orbital amyloidosis Common symptoms Visual impairment Amyloid deposits Lacrimal gland enlargement Yellow nodules on the sag of the eyelids
Primary amyloidosis is a common type, and ocular lesions are part of systemic amyloidosis, the conjunctiva, eyelids and other anatomical parts such as the skin, pharynx, bronchi branches, heart, liver, kidneys, muscles, nerves and blood vessels. There is amyloid deposition, which produces lesions; when the heart is involved, it causes heart failure, when the tongue is invaded, the tongue becomes hypertrophied, making it difficult to swallow and speak; intestinal lesions cause intestinal peristalsis and absorption dysfunction, when the orbital amyloid protein is deposited, It is characterized by waxy yellow nodules on the skin. The blood vessels are fragile due to amyloid deposition around the blood vessels. The eyelids can be bleeding due to minor trauma. The skin involvement of the eyelids is always related to systemic amyloidosis, which is limited to the conjunctiva, lacrimal glands and eyelid lesions. The absence of eyelid skin damage generally indicates no systemic lesions. In these cases, systemic amyloidosis is examined and the results are often negative.
Amyloidosis restricted to the eyelids is very rare. Generally, amyloid deposits first in the conjunctiva and then spreads back around the blood vessels to the fascia sac and eyelids. The most typical lesions are amyloid deposition on the upper and lower iliac conjunctiva and conjunctiva. The lower tissue, the upper epiphyseal plate, the upper levator muscle tendon, the muscles and the Müller muscle, make the upper palate thicker and hypertrophy, causing the ptosis to sag, such as opening the eyelids, showing the fragile and easily bleeding solid scorpion, amyloid deposits The lacrimal gland enlarges the lacrimal gland, invading the muscle to make the extraocular muscle hypertrophy, and the eye movement is limited. If multiple extraocular muscles are involved, it may lead to frozen globe, invasion of the sclera and optic nerve, may affect visual function, and may also invade Other tissues in the eyelids cause eyeballs, eyelid hemorrhage, etc. The mass can compress the tibia to produce optic bone bone compression, but there is no bone destruction.
Secondary amyloidosis is mainly caused by chronic stimulation of infection and inflammation. Plasma cells involved in the inflammatory process may be associated with amyloid deposition. Tuberculosis, rheumatoid arthritis, leprosy and osteomyelitis cause amyloid in the liver. Kidney, spleen and adrenal gland and other parenchymal organs; can also involve both eyelids, but eyelids are rarely violated, local inflammation or infection caused by local amyloid deposits are rare, such as knots, chronic inflammation of the cornea, infection sometimes leads to corneal Amyloid deposition, but it is very rare in the eyelids.
Systemic plasma cell hyperplasia, multiple myeloma, macroglobulinemia, and B-cell lymphoma may cause amyloid deposition in the eyelid due to transient synthesis of immunoglobulin fragments; amyloidosis is also considered aging As part of the autopsy, it is found that the elderly often have amyloidosis.
CT examination showed a solid space-occupying lesion in the lacrimal gland of the iliac crest, the shape of the lesion was irregular, and the uneven density was accompanied by calcification.
Examine
Eyelid amyloidosis
1. Urine examination: urine Bence-Jones protein examination and protein immunoelectrophoresis examination to exclude plasma cell lesions and multiple myeloma.
2. Histopathological examination: amyloidosis is a cell-free, transparent amorphous substance that causes foreign body granuloma reaction. In conventional HE staining, amorphous substances are distributed along the blood vessels, showing eosinophilism. Fibroblasts and inflammatory cells can be seen in the whole lesion, most of which are plasma cells. It can also be seen that characteristic multinuclear foreign body tissue cells are scattered in the lesion area or distributed at the edge of the amyloid deposition zone, in the eosinophil mass. Occasionally, lymphoid follicles, amyloid-positive staining in PAS, Congo red, methyl violet and thioflavin T, showed birefringence and dichroism by polarized light microscopy, and electron microscopy showed that amyloid was cycle-free. , no branched fine fibers.
The chemical composition of amyloid varies from lesion to lesion. The raw material for the synthesis of amyloid comes from blood circulation or local proteins, spontaneously fused into a -flap configuration, and the protein is converted into fiber. The dyeing properties are purely fibrous and rigid. Due to the wrinkle configuration, -flap is present in all starch-deformed lesions. Therefore, the authors suggest that the disease be renamed to beta fibrosis. Protein AL is present in patients with primary systemic amyloidosis and multiple myeloma. May be associated with orbital, vitreous amyloid deposition; also a major protein in primary localized orbital lesions, chemical analysis showing that protein AL represents the N-terminal fragment or all of the light chain, protein of the and immunoglobulin light chain, protein AA is present in patients with secondary amyloidosis. It is a group of heterotypic proteins with a common sequence of 9 amino acids at the N-terminus of the light chain. There is an immunological cross-reactivity between protein AA and protein SAA. Protein AA appears to be blood circulation. The degradation product of the medium protein SAA, under normal conditions, has a low content of protein SAA, which is significantly increased by the emergency response during chronic inflammation and infection.
Other fibrin proteins isolated from amyloid are: protein AFP, present in patients with a certain family genetic predisposition; protein AET is associated with APUD tumors; protein AP is present in all patients with amyloid deposition, with PAS amyloid Related to dyeing.
CT examination: the lacrimal gland was found to be enlarged, the extraocular muscles were thickened or sacral, and the soft tissue mass in the anterior segment of the anterior segment was observed, and spotted calcification was observed.
Diagnosis
Diagnosis and differentiation of orbital amyloidosis
The sputum of the eyelid skin is yellow and lumpy, the conjunctiva of the iliac crest is hard and brittle, and the hemorrhagic sputum has diagnostic value. The CT examination reveals that the lacrimal gland is enlarged, the extraocular muscle is thickened or the sacral cavity, the soft tissue mass in the anterior segment of the anterior segment, or the spotted calcification can be judged. The extent, size and location of the lesion, but the nature of the lesion cannot be determined. To be diagnosed, histopathological examination is required. For patients with plasma cell disease, protein immunoelectrophoresis should be performed. Patients with multiple myeloma should be collected for 24 hours of urine as Bence- Jones protein examination, such as plasma cell myeloma should be considered for bone marrow examination, high peak of IgM in protein immunoelectrophoresis suggests macroglobulinemia.
To distinguish from multiple myeloma and macroglobulinemia, urine Bence-Jones protein examination and protein immunoelectrophoresis should be performed, and attention should be paid to the presence of amyloidosis in other organs of the body.
