Analysis of disease activity and response to treatment in a large Spanish cohort of patients with systemic lupus erythematosus.

OBJECTIVES: The objectives of this paper are to study the impact of disease activity in a large cohort of patients with systemic lupus erythematosus (SLE) and estimate the rate of response to therapies. METHODS: We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients. Data on demographics, disease characteristics: activity (SELENA-SLEDAI), damage, severity, hospitalizations and therapies were collected. Factors associated with refractory disease were identified by logistic regression. RESULTS: A total of 3658 patients (90% female; median SLE duration (interquartile range): 10.4 years (5.3-17.1)) were included. At the time of their last evaluation, 14.7% of the patients had moderate-severe SLE (SELENA-SLEDAI score ≥6). There were 1954 (53.4%) patients who were hospitalized for activity at least once over the course of the disease. At some stage, 84.6% and 78.8% of the patients received glucocorticoids and antimalarials, respectively, and 51.3% of the patients received at least one immunosuppressant. Owing to either toxicity or ineffectiveness, cyclophosphamide was withdrawn in 21.5% of the cases, mycophenolate mofetil in 24.9%, azathioprine in 40.2% and methotrexate in 46.8%. At some stage, 7.3% of the patients received at least one biologic. A total of 898 (24.5%) patients had refractory SLE at some stage. Renal, neuropsychiatric, vasculitic, hematological and musculoskeletal involvement, a younger age at diagnosis and male gender were associated with refractory disease. CONCLUSIONS: A significant percentage of patients have moderately-to-severely active SLE at some stage. Disease activity has a big impact in terms of need for treatment and cause of hospitalization. The effectiveness of the standard therapies for reducing disease activity is clearly insufficient. Some clinical features are associated with refractory SLE.

Evolución y respuesta al tratamiento de un caso de mielitis

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Masa frontotemporal, afectación ocular y nasal / Frontotemporal mass, ocular and nasal involvement

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Tratamiento médico: fármacos de acción lenta, inmunosupresores y terapias combinadas en la artritis reumatoide / Medical therapy: slow acting drugs, immunosuppressive agents and combined therapies in rheumatoid arthritis

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[Sjogren syndrome: clinics and ENT diagnosis]. / Síndrome de Sjögren clínica y diagnóstico O.R.L.

Prospective study covering 38 cases of Sjögren's syndrome (S.S.) diagnosed after early clinical suspicion awaken for xerostomy and xerophtalmy, once excluded those cases which pathology was previously known. Thirty-two cases pertaining to primary malady (P.S.S.) being secondary (S.S.S.) the others. The clinical E.N.T.-symptomatology stand out in our series, because the throat dryness and taste and olfactory alterations. The 53 percent of patients have consulted with other specialists without actualizing the diagnosis. The AA. discuss complementary diagnostic methods (Schirmer's test, rose bengal stain, sialometry, auto-antibodies and biopsy of minor salivary glands) and diagnostic criteria, and fix the otolaryngologist role in the management of these patients.

[Lip biopsy of minor salivary glands in Sjogren's syndrome: histological findings and clinico-pathological correlations]. / Biopsia labial de glándulas salivares menores en el síndrome de Sjögren: hallazgos histológicos y correlaciones clínico-patológicas.

Thirty-seven lip biopsies of minor salivary glands from patients with suspected Sjögren syndrome (SS) and no other known disease were studied. Two cases were excluded for not meeting disease criteria (one had histologic findings of sarcoidosis). Of 35 SS cases, 4 were considered secondary to associated disease. In 24 cases the Daniels technique was used and in 11, an elliptical excision. In 3 elliptical excisions (27.3%) the biopsy did not contain glands (p = 0.002 compared with the Daniels technique). The mean number of glands per biopsy was 7.7 (2-16) with the Daniels technique and 5 (0-15) by elliptical excision (no significant difference). Focal lymphoid sialoadenitis was present in all 32 gland-bearing specimens. Multiple inflammatory foci were found in 28 (87.5%), 8(25%) of which had a diffuse infiltrate. SS presented more frequently as xerostomy in patients with diffuse infiltrate (p = 0.09). In 11 lip biopsies from controls with other diseases of the oral cavity, 7 (63.6%) showed changes suggestive of SS.

["Sacroiliitis" as the initial manifestation of hepatocarcinoma]. / "Sacroileítis" como manifestación inicial de un hepatocarcinoma.

The hepatocellular carcinomas rarely develop in absence of a previous hepatopathy and their first clinical manifestations are not related, in general, to the metastasis located at the bones of the pelvic girdle. We present a multifocal hepatocarcinoma in a 17-year-old woman without known risk factors, whose initial manifestation was a clinical radiological picture suggesting left sacroiliitis. We stress this rare manifestation preceding the diagnosis of neoplasia two years later.