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Biomedical subjects

Josep Font

Publications and source records attributed to Josep Font.

76 records · Page 5Linked to original sources
PubMed2002

Raynaud's phenomenon in primary Sjögren's syndrome. Prevalence and clinical characteristics in a series of 320 patients.

OBJECTIVE: To determine the prevalence of Raynaud's phenomenon (RP) in a large series of patients with primary Sjögren's syndrome (SS) and to identify the clinical and immunological features related to its presence. METHODS: In a cross sectional study, we investigated 320 consecutive patients with primary SS (294 women, 26 men; mean age at onset 60 yrs, range 16-87 yrs). All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Diagnosis of RP in patients with SS was defined as intermittent attacks of digital pallor and/or cyanosis in the absence of any other associated disease or anatomical abnormalities. RESULTS: RP was present in 40 (13%) patients. All were women, with a mean age of 57 yrs (range 18-78). RP preceded onset of sicca symptomatology in 18 (45%) patients. The main triggering factor was exposure to cold, which induced RP in all patients, while emotional stress was a factor in 12 patients, as was job related predisposition in 2. Fifteen (38%) patients required pharmacological treatment with calcium channel blockers (12 patients) or angiotensin converting enzyme inhibitors (2 patients) during colder months, and one patient required treatment with intravenous prostacyclin for ischemic complications. Compared with SS patients without RP, those with RP showed a higher prevalence of articular involvement (50 vs 31%; p = 0.031), cutaneous vasculitis (30 vs 11%; p = 0.003), antinuclear antibodies (95 vs 65%; p < 0.001), anti-Ro/SSA (59 vs 31%; p < 0.001) and anti-La/SSB antibodies (44 vs 20%, p = 0.003). CONCLUSION: We found RP in 13% of patients with primary SS, in almost half of whom RP was the first autoimmune symptomatology. These patients constituted a subset of SS with a higher frequency of some extraglandular features and positive immunological markers. The clinical course of RP seems to be milder in patients with primary SS than in those with other systemic autoimmune diseases such as systemic sclerosis, with no vascular complications and pharmacological treatment needed in only 40% of patients.

Adolescent↗
PubMed2002

Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment.

The term "catastrophic" antiphospholipid syndrome (APS) was introduced to define an accelerated form of APS resulting in multi-organ failure. Although patients with the catastrophic form represent less than 1% of all patients with APS, they are usually in a life-threatening medical situation that requires high clinical awareness. In this article, we propose guidelines for the diagnosis and treatment of patients with catastrophic APS, based on the cumulated published experience of 130 cases with this syndrome. We believe that it is essential that this syndrome should be diagnosed early and treated aggressively. The combination of high doses of heparin and corticosteroids plus intravenous gamma globulins or plasmapheresis is proposed as the treatment of choice in patients with this severe condition. In addition, preventive measures in patients with APS may be effective to avoid the development of catastrophic APS. We recommend that these proposed guidelines be tested in future prospective, collaborative studies to assess clearly the real prevalence and the best therapy for this life-threatening condition.

Journal Article↗