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Granulomatosis with polyangiitis 50-60% have ophthalmologic manifestations, which can be a presenting feature in a minority of patients. Orbital disease is the most common manifestation, and may result in proptosis, restrictive ophthalmopathy, chronic orbital pain, and in chronic cases, orbital retraction syndrome and intractable socket pain.
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. [4] There are several non-specific and vague signs and symptoms associated with amyloidosis. [5] These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and feeling faint with standing. [5]
AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest ...
LECT2 Amyloidosis (ALECT2) is a form of amyloidosis caused by the LECT2 protein. It was found to be the third most common (~3% of total) cause of amyloidosis in a set of more than 4,000 individuals studied at the Mayo Clinic ; the first and second most common forms the disorder were AL amyloidosis and AA amyloidosis , respectively.
Wild-type transthyretin amyloid (WTTA), also known as senile systemic amyloidosis (SSA), [1] is a disease that typically affects the heart and tendons of elderly people. It is caused by the accumulation of a wild-type (that is to say a normal ) protein called transthyretin .
The disorder is primarily associated with eye, skin, and cranial nerve symptoms with the onset of symptoms appearing between the thirties and fifties. [3] The most common characteristic is type II lattice corneal dystrophy with other signs such as polyneuropathy, dermatochalasis, open-angle glaucoma, bilateral progressive facial paralysis, cutis laxa, skin fragility with ecchymosis, facial ...
Differential diagnosis: Chronic inflammatory demyelinating polyradiculoneuropathy, cryoglobulinemia, nodular localized cutaneous amyloidosis, multiple myeloma, monoclonal gammopathy of undetermined significance, waldenstrom hypergammaglobulinemia, scleroderma, raynaud phenomenon, amyloid light chains (AL) amyloidosis, guillain-Barré syndrome ...
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