Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients.

Although clinical signs and symptoms of giant cell arteritis improve promptly after starting glucocorticoid therapy, reports have suggested that the vascular inflammation may persist. To assess the duration and quality of histopathologic changes in treated patients, we prospectively obtained second temporal artery biopsies in patients treated for 3 to 12 months after their first diagnostic biopsy. Forty patients (28 women, 12 men, median age 77 years) agreed to have a second temporal artery biopsy randomly assigned to 3, 6, 9, or 12 months subsequent to the first.

Masses in the membranes.

A 24-year-old woman with systemic lupus erythematosus presented with a 1-year history of painless vision loss in the right eye. Examination was notable for a bitemporal hemianopia. Brain imaging revealed multiple contrast enhancing dural masses, including one along the planum sphenoidale.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

Background: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well.

Methods: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits.

IFN-γ and IL-17: the two faces of T-cell pathology in giant cell arteritis.

Purpose Of Review: Granuloma formation in giant cell arteritis (GCA) emphasizes the role of adaptive immunity and highlights the role of antigen-specific T cells. Recent data demonstrate that at least two separate lineages of CD4 T cells participate in vascular inflammation, providing an important clue that multiple disease instigators may initiate pathogenic immunity.

Recent Finding: IFN-γ-producing Th1 cells and IL-17-producing Th17 cells have been implicated in GCA.

Multiple sclerosis on steroids.

A 57-year-old man developed acute bilateral vision loss clinically consistent with bilateral optic neuritis. Within 1 month of diagnosis, he developed progressive and severe neurologic dysfunction, and repeat MRI demonstrated enhancement of the optic chiasm and optic tracts, as well as a large enhancing lesion within the right parieto-occipital lobe. Stereotactic-guided brain biopsy demonstrated demyelination consistent with multiple sclerosis.

Th17 and Th1 T-cell responses in giant cell arteritis.

Background: In giant cell arteritis (GCA), vasculitic damage of the aorta and its branches is combined with a syndrome of intense systemic inflammation. Therapeutically, glucocorticoids remain the gold standard because they promptly and effectively suppress acute manifestations; however, they fail to eradicate vessel wall infiltrates. The effects of glucocorticoids on the systemic and vascular components of GCA are not understood.

Clinical course and prognosis of trochlear nerve schwannomas.

Purpose: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas.

Design: Nonrandomized retrospective case series.

Participants: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers.

Mitochondrial disorder with OPA1 mutation lacking optic atrophy.

OPA1 is highly expressed in retina and optic nerve. OPA1 mutations were first identified in patients with non-syndromic autosomal dominant optic atrophy. Recently, OPA1 mutations were detected in a multisystemic disorder which has optic atrophy as the core clinical feature and multiple mitochondrial DNA (mtDNA) deletions in muscle.

Toll-like receptors 4 and 5 induce distinct types of vasculitis.

Large vessel vasculitides, such as Takayasu arteritis and giant cell arteritis, affect vital arteries and cause clinical complications by either luminal occlusion or vessel wall destruction. Inflammatory infiltrates, often with granulomatous arrangements, are distributed as a panarteritis throughout all of the artery's wall layers or cluster in the adventitia as a perivasculitis. Factors determining the architecture and compartmentalization of vasculitis are unknown.

T cells in arteritis and atherosclerosis.

Purpose Of Review: Inflammatory vasculopathies, spanning from atherosclerosis to vasculitides, are driven by innate and adaptive immune responses. Instructed by antigen-presenting cells, T cells have unsurpassed skills to orchestrate protective and pathogenic immunity. Pro-inflammatory and anti-inflammatory T cells regulate master pathogenic pathways, providing a framework for novel immunotherapeutic strategies.

Vessel-specific Toll-like receptor profiles in human medium and large arteries.

Background: Inflammatory vasculopathies, ranging from the vasculitides (Takayasu arteritis, giant cell arteritis, and polyarteritis nodosa) to atherosclerosis, display remarkable target tissue tropisms for selected vascular beds. Molecular mechanisms directing wall inflammation to restricted anatomic sites within the vascular tree are not understood. We have examined the ability of 6 different human macrovessels (aorta and subclavian, carotid, mesenteric, iliac, and temporal arteries) to initiate innate and adaptive immune responses by comparing pathogen-sensing and T-cell-stimulatory capacities.

Occurrence of giant cell arteritis...suddenly.

Purpose: To define the kinetics and mechanisms of frank arteritis onset in patients with giant cell arteritis.

Methods: Cytokines were analyzed from tissue of a patient before and after the development of arteritis.

Results: A temporal artery biopsy specimen from a patient with giant cell arteritis showed no pathologic changes on microscopic examination, but there was evidence of early tissue activation of inflammatory markers.

Retinal image stability in head tremor and nystagmus: counterintuitive observations.

Background: Ophthalmoscopy of the fundus in a patient with head tremor is facilitated by image stability, whereas such observation of a patient with nystagmus is made difficult by image movement. Why does this occur?

Methods: We offer an explanation of these observations and derive mathematical proofs for both direct and indirect ophthalmoscopes in various states of ametropia.

Results: By means of image displacement calculations, we have created a graphical display of refractive error versus image displacement, showing zero displacement in emmetropia in head tremor and exaggerated displacement during nystagmus, regardless of the refractive status of the eye.

Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial.

Objective: Glucocorticoid (GC) therapy for giant cell arteritis (GCA) is effective but requires prolonged administration, resulting in adverse side effects. The goal of the current study was to test the hypothesis that induction treatment with high-dose pulse intravenous (IV) methylprednisolone permits a shorter course of therapy.

Methods: Twenty-seven patients with biopsy-proven GCA were enrolled in a randomized, double-blind, placebo-controlled study to receive IV methylprednisolone (15 mg/kg of ideal body weight/day) or IV saline for 3 consecutive days.

Oprelvekin-associated bilateral optic disk edema.

Purpose: To report a case of bilateral optic disk edema in a patient taking oprelvekin for radioimmunotherapy-induced thrombocytopenia.

Design: Observational case report.

Methods: A 38-year-old man with a history of relapsed non-Hodgkins follicular lymphoma complained of bilateral loss of vision following oprelvekin therapy for thrombocytopenia.

Initiation of glucocorticoid therapy: before or after temporal artery biopsy?

Objective: To identify clinical findings in patients with suspected giant cell arteritis (GCA) that may help clinicians decide when to initiate glucocorticoid therapy.

Patients And Methods: Medical diagnostic codes and surgical indexing were used to identify all patients who had temporal artery biopsy at the Mayo Clinic in Rochester, Minn, between January 1, 1988, and December 31, 1997. Patient medical records were abstracted for pertinent clinical data, glucocorticoid use, and final diagnoses.

Trapping of misdirected dendritic cells in the granulomatous lesions of giant cell arteritis.

Immature dendritic cells (DCs) are scattered throughout peripheral tissues and act as sentinels that sample the antigenic environment. After activation, they modify their chemokine receptor profile and migrate toward lymphoid tissues. On arrival, they have matured into chemokine-producing DCs that express co-stimulatory molecules and can prime naive T cells.

Reactive nitrogen intermediates in giant cell arteritis: selective nitration of neocapillaries.

Arterial wall damage in giant cell arteritis (GCA) is mediated by several different macrophage effector functions, including the production of metalloproteinases and lipid peroxidation. Tissue-invading macrophages also express nitric oxide synthase (NOS)-2, but it is not known whether nitric oxide-related mechanisms contribute to the disease process. Nitric oxide can form nitrating agents, including peroxynitrite, a nitric oxide congener formed in the presence of reactive oxygen intermediates.