Ocular involvement in giant cell arteritis

Giant cell arteritis (GCA) is a systemic disease with a wide spectrum of clinical manifestations. Severe visual loss is the most frequent serious complication. This article reviews current ideas about GCA and in particular its ocular manifestations.     

Etiopathogenesis of giant cell arteritis

Giant cell arteritis is a vasculitis of large and medium size arteries, especially those of the aortic arch with an extracranial distribution, but also the aorta and some of its larger branches. It is characterised by the presence of mononuclear inflammatory infiltrates close to the internal elastic lamina formed by lymphocytes and macrophages, which in slightly more than 50% of the cases contain multinucleate giant cells. The morbidity associated with this disease is related to phenomena of distal ischemia to the luminal stenosis of the inflamed arteries, and to a lesser extent to the formation of aneurisms due to the weakening of the arterial wall. With an unknown aetiology, its pathogenesis is immune through the migration and location of gamma-INF -producing T cells in the adventitia of the inflamed arteries, it being assumed that this is the place of immune stimulation by a still unidentified antigen. The recruitment and activation of macrophages by this cytokine is one of the most important points of its pathogenesis. The destruction by these of the arterial elastic tissue is a relevant phenomenon, as is the production of other factors promoting neoangiogenesis and a proliferation of neointime, responsible through obliterating light for the ischemic manifestations of the disease. The process is accompanied by an important systemic repercussion characterised by a strong reaction of acute phase and general but barely specific symptoms of disease. On the other hand, an important percentage of patients show a clinical picture of polymyalgia rheumatica, an entity that has a historical and controversial relationship to this arteritis. In recent years important contributions have been made to the understanding of the immune mechanisms involved in its pathogenesis.     

Cytomegalovirus infection in an immunocompetent patient complicated with pulmonary embolism and mimicking giant cell arteritis associated with polymyalgia rheumatica

Cytomegalovirus infection is generally asymptomatic in immunocompetent patients but can also provide a wide spectrum of clinical and biological signs. We report the case of a cytomegalovirus infection in an immunocompetent adult mimicking giant cell arteritis associated with polymyalgia rheumatica and complicated with pulmonary embolism. We review and discuss the venous prothrombic properties of CMV in immunocompetent patients.     

Diagnosis and prognosis of polymyalgia rheumatica and giant cell arteritis

Retrospectively the data were examined of 69 patients with polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA), of whom 62 were treated with corticosteroids. The clinical and laboratory data, and the outcome of temporal artery biopsy were compared. In addition the relation between the course of the disease and survival was investigated. PMR and GCA are closely related syndromes with a multiform clinical presentation. There even is a group of patients with merely systemic symptoms, without specific signs of PMR or GCA. Concerning the course of the disease patients can be divided into groups with a shorter and longer period of disease activity; patients with a smooth stable remission and a chronic disease. The former group shows a significantly better five-year survival. At the time of diagnosis no reliable prediction of the course of the disease is possible.     

Two coronary artery fistulae in a patient with temporal arteritis and chest pain.

A 59-year-old woman with a history of cardiac sounding chest pain was investigated by coronary arteriography. Two unsuspected fistulae were found. The fistulae originated from the distal right coronary artery and the diagonal branch of the left coronary artery. Both drained into the left ventricle. She was also found to have temporal arteritis, treatment of which abolished the chest pain.     

Atypical mole syndrome and congenital giant naevus in a patient with celiac disease

We describe a case of a 28-year-old woman affected by celiac disease (CD) associated with rare multiple disorders of the cutaneous pigmentary system: atypical mole syndrome and congenital giant naevus. Some other rare skin lesions have been reported in association with celiac disease such as cutaneous sarcoidosic granuloma, bullous pemphigoid, ichthyosis, alopecia areata, erythema elevatum diutinum, sclero-atrophic lichen and primary cutaneous amyloidosis. This is the 1(st) report concerning celiac disease and congenital disorders of the pigmentary system.