Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Both unilateral proptosis (2 cases) and radiological evidence of orbital inflammatory pseudotumor in the absence of exophthalmos (1 case) have been separately described as presenting signs of temporal arteritis. We report a patient presenting with bilateral exophthalmos associated with CT and MRI signs of orbital inflammation, who had biopsy-proven temporal arteritis. Our case is unique in view of the association of the above clinical and radiological features, and the bilateral involvement of orbital tissues.
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PMID:Orbital pseudotumor as a presenting sign of temporal arteritis. 755 67

A 75-year-old woman developed acute loss of vision in the OD, ipsilateral periocular pain, an afferent pupillary defect, sectoral optic disc edema, and later ipsilateral proptosis and an intraconal mass. She denied any symptoms of temporal arteritis, and a sedimentation rate was normal. Orbital biopsy demonstrated chronic granulomatous inflammation with perivasculitis. A temporal artery biopsy disclosed findings consistent with temporal arteritis. Following 2000 cGy of external beam radiation, her visual function and orbitopathy completely resolved. This unusual presentation of orbital inflammation in association with temporal arteritis demonstrates that pathologic findings of temporal arteritis may be clinically nonspecific and that external beam radiation may be an effective therapy in this setting.
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PMID:Radiosensitive orbital inflammation associated with temporal arteritis. 1278 21

A 71-year-old man visited our clinic with a 3-day history of severe throbbing headache and 1-day history of horizontal diplopia. He had had jaw claudication and pain in the neck and shoulder several days previously. His right eye was slightly esotropic and did not move laterally. There was no blepharoptosis, proptosis, lid edema, or conjunctival injection. The pupils were unremarkable. The remainder of the cranial nerve functions was intact. There was no limb weakness or sensory impairment. Superficial temporal arteries were swollen and tender on both sides. Laboratory examination showed elevated CRP level and high erythrocyte sedimentation rate. Cranial MR images were unremarkable. The cerebrospinal fluid was acellular with 45 mg/dl of protein. A diagnosis of temporal arteritis was made. Treatment with 50 mg of prednisolone brought about prompt disappearance of the headache. Right ocular movement fully recovered in 10 days. Temporal artery biopsy findings and response to corticosteroid were consistent with temporal arteritis. The motility pattern of the right eye was consistent with complete abducens nerve palsy, which is a rare manifestation of temporal arteritis. Although temporal arteritis is a rare cause of ophthalmoplegia in the elderly patients, swift diagnosis and treatment is necessary to avoid blindness.
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PMID:[Temporal arteritis presenting with headache and abducens nerve palsy. Report of a case]. 1771 Aug 91

Giant cell arteritis (GCA) often presents with symptoms of headache, jaw claudication, polymyalgia rheumatica, and blurred vision. GCA is relatively rare and may have atypical manifestations in Asians, including multiple cranial nerve palsy and reversible proptosis. A high suspicion of GCA is suggested when any older Asian suffers from headache that is new-onset or different from the previous pattern, even without other typical manifestations of GCA.
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PMID:Giant cell arteritis with multiple cranial nerve palsy and reversible proptosis: a case report. 1805 56

Inflammatory orbital pseudotumor is often associated with rheumatologic disorders. It has been reported commonly with ANCA-associated vasculitides, especially granulomatosis with polyangiitis (Wegener's granulomatosis). There are also a few cases of large vessel vasculitis such as giant cell arteritis and Behcet's disease. Here, we report a patient with undiagnosed Takayasu arteritis presenting with proptosis and diplopia, with later diagnosis of an inflammatory pseudotumor of the orbit. In this case, we believe extensive involvement of blood vessels, including bilateral pulmonary artery stenosis, and elevated inflammatory markers that show disease activity may be related to pseudotumor formation in Takayasu arteritis. Since this is an unusual and unreported presentation of the disease, better estimation of a causal relationship may be possible in the future with further information. In conclusion, although uncommon, this case highlights that orbital pseudotumor may be an important finding in Takayasu arteritis. For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides.
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PMID:Bilateral orbital pseudotumor in a patient with Takayasu arteritis: a case report and review of the literature. 2684 15

A 63-year-old man presented with a 2-month history of progressive right-sided exophthalmos, painful ophthalmoplegia and fevers. As more features developed, he was diagnosed with giant cell arteritis, then Tolosa-Hunt syndrome, and transiently responded to corticosteroids. A bland cerebrospinal fluid and highly metabolically active brain (18F)-fluoro-D-glucose-positron emission tomography suggested lymphoma. Biopsy of the mass showed sulphur granules with Gram-positive filamentous bacteria with Actinomyces-like colonies. Actinomyces cavernous sinus infections are rare and indolent. They often mimic non-infective causes including other inflammatory and infiltrative conditions, vascular and neoplastic causes, particularly lymphoma. Clinicians should consider infective cavernous sinus syndromes in people with a fluctuating painful ophthalmoplegia that responds poorly to corticosteroids. The term Tolosa-Hunt syndrome is problematic and should be retired or used only with reservation.
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PMID:Actinomyces cavernous sinus infection: a case and systematic literature review. 2965 Jun 38

Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow. Lymph nodes are typically spared. The clinical picture depends on the specific organ involvement making the correct diagnosis very difficult. Here, we report a case of intravascular large B-cell lymphoma diagnosed postmortem on a 69-year-old African-American male who presented with unilateral proptosis and visual loss. An initial diagnosis of temporal arteritis was made and the patient received corticosteroids. However, the patient developed multiorgan failure and expired. On autopsy, there was disseminated intravascular lymphoma involving predominantly vessels within the heart, kidneys, liver, stomach, lungs, adrenal glands, small intestine, bladder, thyroid, and brain. Interestingly, there was also partial involvement of the retroperitoneal lymph nodes which is an unusual presentation in this disorder. Immunohistochemical staining showed that the lymphoma cells were positive for CD20, indicating B-cell phenotype. This case supports the "mimicking nature" of this rare entity with an unusual presentation with proptosis and visual loss, simulating temporal arteritis and a rare involvement of the retroperitoneal lymph nodes. The presentation of intravascular large B-cell lymphoma can vary, and the key to diagnosis is dependent on histopathology and immunohistochemistry. Increased awareness, early tissue diagnosis, and prompt chemotherapy are crucial for this otherwise lethal disease.
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PMID:Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis. 2985 38