Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cogan's syndrome is an uncommon entity of nonsyphilitic interstitial keratitis with vestibulo-auditory disturbances. Although it is unusual, Cogan's syndrome should be considered in the differential diagnosis of patients with sudden hearing loss, even when they lack ophthalmologic symptoms. Systemic manifestations are not uncommon and, along with serologic and hematologic abnormalities, may help in making the diagnosis, which requires a high index of suspicion. Treatment with steroids has largely been based on symptoms. We suggest using the C-reactive protein level as a monitor of subclinical disease activity; it is therefore beneficial in the adjustment of steroid therapy. We have described the case of a 41-year-old woman who sought treatment for an upper respiratory infection syndrome and severe vertigo. Evaluation included hematologic and serologic studies, lumbar puncture, and CT and MRI scans. Abnormal findings consisted of an elevated white blood cell count and an ESR of 112 mm/hr. Six days later, profound, bilateral sensorineural hearing loss developed suddenly. Intensive corticosteroid and vasodilator therapies were instituted, but there was no improvement in hearing levels. Ten days later eye pain and redness developed, and ophthalmologic evaluation revealed an interstitial keratitis consistent with Cogan's syndrome. Steroid eye drops and oral prednisone therapy promptly relieved the eye symptoms. Steroid tapering was associated with diffuse joint pain and swelling consistent with a systemic vasculitis. After rheumatologic consultation, steroid dosage was titrated to the CRP level and ESR, and vasculitic symptoms resolved. Hearing levels did not improve, and the patient had cochlear implantation. Thirteen cases of bilateral sudden deafness due to Cogan's syndrome have been reported previously. This is the first case in which there were no immediate eye symptoms.
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PMID:Cogan's syndrome manifesting as sudden bilateral deafness: diagnosis and management. 836 55

A middle-aged woman who experienced recurrent episodes of unilateral vision loss and eye pain. On presentation, magnetic resonance imaging (MRI) demonstrated left optic nerve enhancement with patchy hyperintensities in the white matter of both frontal lobes and ill-defined enhancement in a lenticulostriate distribution. Ophthalmologic examination revealed left optic disc edema with a macular scar consistent with neuroretinitis. Her subsequent clinical course was notable for 2 episodes of painful vision loss, without associated neurologic symptoms, which resolved with intravenous and oral steroids. More than 1 year after her initial presentation, the patient developed right facial weakness and slurred speech, and shortly thereafter suffered a fatal intracerebral hemorrhage. Histopathology on autopsy confirmed a diagnosis of primary angiitis of the central nervous system (PACNS). This is an unusual case of PACNS presenting with recurrent unilateral optic neuritis. The vascular enhancement pattern on MRI suggesting inflamed cerebral blood vessels is a rarely described pattern, which likely reflects intracerebral extension of the ocular pathology. The combination of neuroretinitis and perivascular MRI enhancement pattern may represent a subtype of PACNS.
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PMID:Primary angiitis of the central nervous system presenting as unilateral optic neuritis. 2540 82

Granulomatosis with polyangiitis (GPA) sometimes involves the eye orbit; however, choroidal involvements in GPA had been rarely reported. We report a rare case presenting with a choroidal mass in an 83-year-old Japanese woman who presented with left eye pain. Diagnostic biopsy revealed necrotizing vasculitis with infiltrates of inflammatory cells. Diagnosis was localized granulomatosis with polyangiitis. Combined treatments with corticosteroid plus azathioprine resolved the choroidal mass region. Although treatment with corticosteroid and immunosuppressive agents improves the prognosis of the disease, ocular morbidity is still well recognized. Clinicians should consider a differential diagnosis of GPA in patients with inflammatory choroidal tumors.
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PMID:Granulomatosis with polyangiitis presenting as a choroidal tumor. 2594 41

A 20-year-old woman presented with headache, decreased vision, eye pain, and urinary retention. During her clinical course, visual acuity declined to 20/800, right eye, and 20/50, left eye, associated with bilateral optic disc edema. Brain magnetic resonance imaging revealed enhancement of the leptomeninges, right optic nerve, and right side of the optic chiasm. Extensive evaluation of the central nervous system (CNS) for an infectious cause was negative. Brain biopsy showed a pattern consistent with vasculitis. The patient was treated with prednisone and cyclophosphamide, resulting in improvement of her vision and systemic symptoms. Primary CNS vasculitis is a rare condition that may affect the anterior visual pathways.
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PMID:Primary Central Nervous System Vasculitis With Optic Nerve Involvement. 2669 42

Fungal infection should be considered in the differential diagnosis of a pituitary or sellar mass, albeit fungal infections involving the pituitary gland and sella are a rare occurrence. We report a case of Aspergillus infection involving the pituitary gland and sellar region discovered in a 74-year-old man. The patient had a history of hypertension, chronic renal disease, autoimmune hemolytic anemia and presented with right eye pain, headaches and worsening hemiparesis. Imaging studies revealed a right internal carotid artery occlusion and an acute right pontine stroke along with smaller infarcts in the right middle cerebral artery distribution. Clinically, the patient was thought to have vasculitis. An infectious etiology was not identified. He developed respiratory distress and died. At autopsy, necrotizing meningitis was discovered. A predominantly chronic inflammatory cell infiltrate consisting of benign-appearing lymphocytes, plasma cells and macrophages was accompanied by acute angle branching, angioinvasive hyphae which were highlighted on Gomori methenamine silver staining and were morphologically consistent with Aspergillus species. In previously reported cases of Aspergillus infection involving the pituitary or sella, most presented with headaches or impaired vision and were not immunocompromised. A transsphenoidal surgical approach is recommended in suspected cases in order to minimize the risk of dissemination of the infection. Some patients have responded well to antifungal medications once diagnosed.
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PMID:Pituitary aspergillus infection. 2689 7

A 74-year-old man presented with right eye pain, swelling, photophobia, and vision loss 1 month following placement of an intravitreal dexamethasone implant (Ozurdex; Allergan, Irvine, CA) for intraocular inflammation refractory to topical therapy. Clinical examination showed visual acuity of 20/400, anterior chamber cell, vitritis, retinal vasculitis, and extensive retinitis. A vitreous biopsy was performed followed by intravitreal injections of vancomycin, ceftazidime, ganciclovir, and clindamycin. Polymerase chain-reaction testing was positive for Toxoplasma gondii. After a course of systemic therapy, intraocular inflammation subsided but visual acuity remained poor. This is the first case to the authors' knowledge of reactivation of T. gondii following intravitreal implantation of dexamethasone. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1050-1052.].
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PMID:Reactivation of Latent Toxoplasmosis Following Dexamethasone Implant Injection. 2784