[Sarcoid myopathy. Report of two cases and review of the bibliography]. / Miopatía sarcoidea. Presentación de dos casos y revisión de la bibliografía.

INTRODUCTION: 5% of patients with sarcoidosis are affected by neurological complications, of which myopathy is one of the least frequent. We report the clinical course and therapeutic response of two patients with sarcoid myopathy. CASE REPORTS: We observed two females aged 63 and 55 who were previously diagnosed with sarcoidosis, which in one case was cutaneous and ophthalmic and in the other pulmonary. Both were asymptomatic from a systemic point of view when they visited the Neurology department. Both patients developed chronic myopathy (with a history of 9 months and 6 years), which was predominantly proximal, painful and both of them had normal or slightly high levels of creatine phosphokinase. The EMG was clearly myopathic in the two patients. A muscle biopsy showed a mononuclear-cell infiltrate with the formation of non-caseating granulomas. Both patients were treated with prednisone, although the therapeutic response was different in each case. One of the patients showed an important improvement in just a few days, while in the other case the disease remained stable, despite adding, first, azathioprine and later methotrexate to the treatment. CONCLUSIONS: Sarcoidosis can cause chronic, predominantly proximal, myopathy, and chiefly affects females over 50 years of age. The therapeutic response to steroids and immunosuppressants varies from case to case.

[Rhabdomyolysis and lumbosacral plexopathy in intravenous drug addict: report of a case]. / Rabdomiolisis y plexopatía lumbosacra en paciente adicto a drogas por vía parenteral: a propósito de un caso.

There are several neuromuscular complications in the intravenous heroin addict (IHA). Someone may be due to direct toxic effect of the substance, but other ones may be associated to abuser's typical diseases (i.e. HIV infection). We present a 27 year-old IHA patient, HIV positive, that develop acute rhabdomyolisis with severe neuromuscular involvement, and consistent clinical and electrodiagnostic features of lumbosacral plexus neuropathy, forteen hours after an heroin inyection. Thirty months later, the patient is severely disabled, but her initial painfull and paretic picture have improved. The association of rhabdomyolisis-lumbosacral plexopathy (RLPS) is ocasionally reported. It has been proposed that RLSP is etiologically related to mecanic, toxic and immunologic factors.

[Class I and II HLA antigens in patients with systemic lupus erythematosus in the south of Spain]. / Antígenos HLA de clase I y II en pacientes con lupus eritematoso sistémico en el sur de España.

BACKGROUND: To establish the relation between class I and II HLA antigens, systemic lupus erythematosus (SLE), autoantibodies production, and clinical manifestations in the south of Spain (Málaga). METHODS: In a regional hospital we undertook a case-control study with a consecutive sample of 104 patients with SLE who fulfilled at least 4 criteria of ARA. Three hundred and twenty-eight local controls with no apparent pathology were included for comparison. We evaluated clinical and analytical aspects about multisystem autoimmune disease. HLA typing was serologically determined. RESULTS: Univariate analysis showed a relation between SLE and the specificities B8 (21% of patients vs 10% of controls, p = 0.005; RR = 2.3), DR3 (36% vs 20%, p = 0.0006; RR = 2.5), DRw52 (69% vs 49%, p = 0.001; RR = 2.3), and DQ2 (49% vs 36%, p = 0.0150; RR = 1.7). However, in logistic regression multivariate analysis, there was a confounding effect between DR3 and DRw52, and it could be that only this specificity, HLA-DRw52 (RR = 2.0; 95% CI: 1.1-4.0), and of lesser degree B8 (RR = 1.9; 95% CI: 0.9-4.4), are really associated with SLE. Also, in multivariate analysis, DR6 showed a negative association (5% vs 25%, p = 0.011; RR = 4.2; 95% CI: 1.5-17.2) with anti-U1RNP, while DRw52 showed a negative association with IgG-aCL (50% vs 85%, p = 0.019; RR = 0.21; 95% CI: 0.06-0.76). Furthermore, DQ2/DQ6 showed positive association with anti-SSA/Ro antibodies (50% vs 24%; p = 0.046; RR = 3.0; 95% CI: 1.0-9.0). There were also several associations between clinical manifestations and HLA. The specificities DR and DRw53 were almost always risk factors, but only DR5 was a protector for renal lesion. DRw52 and DQ specificities were always protectors when they were associated with some clinical manifestations. Isolated DR3 antigen, is not associated with any of the above-mentioned manifestations. CONCLUSIONS: The previously described relation between SLE and the antigen DR3 is confirmed, but this association could be a result of the presence of DRw52 specificity in patients, that is in linkage disequilibrium with DR3.

[Immunogenetics of the Sjogren's syndrome in southern Spain]. / Inmunogenética del síndrome de Sjögren en el sur de España.

To establish the relation between HLA antigens, Primary Sjögren Syndrome (PSS) and autoantibodies production, in our geographical area, we undertook a case-control study with a consecutive sample of 30 patients with PSS (Fox's criteria) attending in a reference hospital. Two hundred and sixty-four local controls with no apparent pathology were included for comparison. In patients we evaluated clinical and analytical aspects about multisystem autoimmune disease. Anti-SSA/Ro and -SSB/La autoantibodies were determined by double immunodiffusion. HLA typing was serologically determined. In logistic regression multivariate analysis, there were significant association between PSS and specificities HLA-Cw7 (73% in cases, versus 35% in controls; RR = 8.0; 95% CI: 23.2-2.7), HLA-DR3 (63% vs 20%; RR = 3.4; 95% CI: 9.5-1.4) and HL-DR11 (43% vs 13%; RR = 4.1; 95% CI: 12.0-1.4). In patients, the anti-SSA/Ro autoantibodies production were associated with HLA-DR3 antigen (83% vs 25%; RR = 6./; 95% CI: 1.3-34.2). All HLA-DQ2/DQ6 heterozygotes patients (8 cases) had anti-SSA/Ro autoantibodies, versus only one half of the remainder (p = 0.029; RR = 6.3). In anti-SSA/Ro negative patients there weren't association with HLA-DR3 antigen (33% vs 20%). HLA-DR3 were associated with the presence of anti-SSB/La autoantibodies, but there wasn't signification (p = 0.081). We conclude that our patients with PSS present association with HLA-DR11 specificity, that it's a risk factor for the disease development. HLA-DR3 would determined the anti-SSA/Ro autoantibodies, and maybe also anti-SSB/La autoantibodies, production. Furthermore, HLA-DQ2/DQ6 heterozygosity would determined immune response to SSA/Ro autoantigen.