Silent versus cranial giant cell arteritis. Initial presentation and outcome of 50 biopsy-proven cases.

BACKGROUND: The objective of the present study was to compare the silent form of giant cell arteritis (GCA) to the classic cephalic form of the disease. METHODS: We conducted a retrospective study based on a chart review of 50 consecutive, biopsy-proven GCA, recorded at a department of internal medicine. We sought to distinguish a silent form, defined by a prolonged inflammatory syndrome or fever of unknown origin with the absence of cephalic signs, polymyalgia rheumatica, or large artery involvement, from an overt "classic" cranial temporal arteritis. RESULTS: The prevalence of the silent form of GCA was 46% in our study. Abnormal temporal arteries were more frequent in the cephalic group. The silent GCA group had higher C-reactive protein levels (p<0.05), a higher platelet count (p<0.05), and lower serum albumin (p<0.05). There was no significant difference in temporal artery specimens in the two groups. Clinical relapses tended to be more frequent, and patients free of corticosteroids tended to be less frequent, in the cephalic group, though the difference was not statistically significant. CONCLUSIONS: The silent and cephalic forms of GCA could have distinct clinical and biological patterns and different outcomes. The limitation of our study was its retrospective design. Further studies are required to determine if this distinction is useful in treating GCA patients.

Temporal arteritis associated with systemic necrotizing vasculitis.

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.

Antisynthetase syndrome.

Seven autoantibodies directed against synthetases have been identified to date, the best known being anti-Jo1. Synthetases play a vital role in protein synthesis by catalyzing the acetylation of transfer RNAs (tRNAs). The most common form of antisynthetase syndrome is characterized by anti-Jo1 production, interstitial lung disease (ILD), inflammatory muscle disease, and, in many cases, fever, polyarthritis, Raynaud's phenomenon, and thick cracked skin on the fingers (mechanic's hands). The interstitial lung disease is generally of the "usual interstitial pneumopathy" type and shares similarities with idiopathic pulmonary fibrosis or scleroderma-related pulmonary disease. It governs the prognosis of the disease, being associated with an excess mortality rate of about 40%. The pathogenic mechanisms underlying antisynthetase syndrome remain unknown but may involve cell-mediated immunity. The treatment is not standardized. The ILD responds to glucocorticoids in some patients but requires other immunosuppressant drugs in others.

[Kikuchi-Fujimoto disease: two cases]. / Maladie de Kikuchi-Fujimoto. Deux nouvelles observations.

Kikuchi-Fujimoto disease, or necrotizing histiocyte lymphadenitis, is a rare anatomo-clinical entity of unknown cause. The patients present painful cervical lymph nodes, fever, and a flu-like syndrome, but the spontaneous course is usually favorable in six months. We observed two cases within six months in our unit. Both patients improved rapidly after diagnostic adenectomy.

[Antisynthetase syndrome. Three cases and a review of the literature]. / Syndrome des anti-synthétases. A propos de 3 observations et revue de la littérature.

Antisynthetase syndrome belongs to the idiopathic myositis group which includes pulmonary interstitial disease, arthritis, Raynaud's phenomenon, and mechanic's hands , associated with the anti-Jo1 antibody. We report three cases of antisynthetase syndrome, and review the clinical characteristics, and prognosis factors dominated by interstitial pneumonia.