[Caution with the use of dexamethasone in patients with COVID-19 in its initial phases]. / Precaución con el uso de dexametasona en pacientes con COVID-19 en sus fases iniciales.

INTRODUCTION: The effect of dexamethasone in the initial phase of infection by SARS-CoV-2 and its influence on COVID-19 is not well defined. We describe clinical-radiological characteristics, the cytokine storm parameters, and the clinical evolution of a series of patients treated with dexamethasone in the disease's initial phase. METHOD: A study of 8 patients who received dexamethasone before the development of COVID-19. We evaluate clinical variables, imaging tests, cytokine release parameters, treatment used and patient evolution. RESULTS: All patients received a 6 mg/day dose with a mean duration of 4.5 days before admission. High resolution computed tomography (HRCT) revealed that most of them presented a severe extension; most patients had a slightly elevated level of cytokine release parameters. Three patients required high-flow oxygen therapy due to respiratory failure; none required orotracheal intubation or died. CONCLUSION: Dexamethasone in the early stages of SARS-CoV-2 infection appears to be associated with severe COVID-19.

Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.

OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

Antiphospholipid syndrome, antiphospholipid antibodies and solid organ transplantation.

Antiphospholipid syndrome is considered a high risk factor for any kind of surgery. Considering that all solid organ transplants are critically dependent on the patency of vascular anastomosis, there is much concern about the consequences this pro-thrombotic condition may have on transplantation. Relatively little information is available in the literature assessing the real risk that antiphospholipid syndrome or the presence of antiphospholipid antibodies represent in solid organ transplantation. The aim of this article is to review the literature related to transplantation of solid organs in patients diagnosed with antiphospholipid syndrome or patients with positive antiphospholipid antibodies.

Pyoderma gangrenosum and systemic lupus erythematosus: a report of five cases and review of the literature.

Pyoderma gangrenosum (PG) is an uncommon, distinctive cutaneous ulceration which is usually idiopathic, but may be associated with many systemic disorders. The etiopathogenesis of PG is still not well understood. PG is part of the spectrum of the neutrophilic dermatoses and it has been proposed as a prototype of cutaneous autoinflammatory disease. PG usually has a good outcome under immunosuppressive treatment. Although PG has been associated with several systemic diseases, it has rarely been reported in association with systemic lupus erythematosus (SLE). In this article we report five cases of SLE-related PG and review the literature. Our findings support the possible relationship between active SLE and PG, although the mechanism remains unclear. Clinical manifestations, used treatments and outcomes of SLE-related PG do not differ from the described for the general population.

Impaired sexual function in women with systemic lupus erythematosus: a cross-sectional study.

OBJECTIVE: The objectives of this paper are to compare sexual function and distress in women with systemic lupus erythematosus (SLE) and in healthy controls; to determine the association between disease characteristics, quality of life, psychopathology and sexual function; and to compare sexual function and distress of women according to age (reproductive and nonreproductive-age women). METHODS: We conducted a cross-sectional study of 120 participants; 65 women had SLE (aged 18-65), and 55 were healthy, age-matched controls. The assessment included the Female Sexual Function Index (FSFI), Symptom Checklist-90-Revised (SCL-90-R), Short Form 36 health survey (SF-36), socio-demographic characteristics and the Systemic Lupus International Collaborating Clinics (SLICC) and SLE Disease Activity Index (SLEDAI) in SLE patients only. RESULTS: Of 65 eligible patients with SLE, 61 (94%) responded; of 55 control subjects, 53 (96%) responded. The FSFI total score and subscale scores for desire, arousal, lubrication, orgasm and pain were significantly lower in patients with SLE. More somatization, obsessive-compulsive symptoms, interpersonal sensitivity, depression, anxiety, hostility, phobia, paranoid ideation, psychoticism, the Positive Symptom Total (PST), Positive Score Discomfort Index (PSDI), the use of psychotropic drugs, general health, vitality, social function, emotional role and mental health were significantly associated with changes in the patient group's sexuality. Multivariate analysis indicated that depression, PSDI and vitality were the variables significantly associated with low sexual function in patients with SLE. CONCLUSIONS: Women with SLE reported significantly impaired sexual function compared with healthy controls. Impaired sexual function was associated with somatization, obsessive-compulsive behavior, interpersonal sensitivity, depression, hostility, paranoid ideation, psychoticism, PST, higher scores in the PSDI subscale, vitality, social functioning and mental health. These results indicate that, in daily practice, inquiring about sexuality and quality of life and screening for psychopathology are important for every patient with SLE, irrespective of their clinical characteristics.

The autoimmune disease-associated IL2RA locus is involved in the clinical manifestations of systemic sclerosis.

Regulatory T cells (T(regs)) are crucial in the maintenance of the immune tolerance and seem to have an important role in systemic sclerosis (SSc). The interleukin 2 receptor α (IL2RA) is an important T(reg) marker, and polymorphisms of IL2RA gene are associated with a number of autoimmune diseases. Therefore, we aimed to investigate for the first time the association of the IL2RA locus in SSc. For this purpose, a total of 3023 SSc patients and 2735 matched healthy controls, from six European Caucasian cohorts, were genotyped for the IL2RA gene variants rs11594656, rs2104286 and rs12722495 using the TaqMan allelic discrimination technology. The overall meta-analysis reached statistical significance when the three polymorphisms were tested for association with SSc, the limited subtype (lcSSc) and anti-centromere auto-antibodies (ACAs). However, no significant P-values were obtained when the ACA-positive patients were removed from the SSc and lcSSc groups, suggesting that these associations rely on ACA positivity. The strongest association signal with ACA production was detected for rs2104286 (P(FDR)=2.07 × 10(-4), odds ratio=1.30 (1.14-1.47)). The associations of rs11594656 and rs12722495 were lost after conditioning to rs2104286, and allelic combination tests did not evidence a combined effect, indicating that rs2104286 best described the association between IL2RA and ACA presence in SSc.

No evidence of association between functional polymorphisms located within IL6R and IL6ST genes and systemic sclerosis.

Systemic sclerosis (SSc) is a complex autoimmune disease which genetic component has not been yet completely understood. IL6 encodes a cytokine with a crucial role in the development of autoimmunity and fibrosis and its actions mainly are controlled by IL-6 receptor (IL-6R). We aimed to investigate whether the functional genetic variants rs8192284 and rs2228044 previously associated with several autoimmune diseases, located within the IL-6 receptor (IL-6R) subunits IL6R and IL6ST genes, respectively, are involved in the susceptibility to SSc and/or its major clinical subphenotypes. A Spanish cohort including 1013 SSc patients and 1375 controls was genotyped using the TaqMan® allelic discrimination technology. SSc patients were subdivided according to the major clinical forms, autoantibody status and presence of fibrotic lung affection. Our data showed no influence of the selected variants in global SSc susceptibility (rs8192284: P=0.67, odds ratios (OR)=0.98; rs2228044: P=0.99, OR=1.00). Similarly, the clinical/autoantibody subphenotype analyses did not yielded significant results. Our data suggest that the analyzed polymorphisms may not play a significant role in the SSc susceptibility.

Caution with the use of dexamethasone in patients with COVID-19 in its initial phases.

INTRODUCTION: The effect of dexamethasone in the initial phase of infection by SARS-CoV-2 and its influence on COVID-19 is not well defined. We describe clinical-radiological characteristics, the cytokine storm parameters, and the clinical evolution of a series of patients treated with dexamethasone in the disease's initial phase. METHOD: A study of 8 patients who received dexamethasone before the development of COVID-19. We evaluate clinical variables, imaging tests, cytokine release parameters, treatment used and patient evolution. RESULTS: All patients received a 6 mg/day dose with a mean duration of 4.5 days before admission. High resolution computed tomography (HRCT) revealed that most of them presented a severe extension; most patients had a slightly elevated level of cytokine release parameters. Three patients required high-flow oxygen therapy due to respiratory failure; none required orotracheal intubation or died. CONCLUSION: Dexamethasone in the early stages of SARS-CoV-2 infection appears to be associated with severe COVID-19.

Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature.

OBJECTIVE: Rituximab is an anti-CD20 monoclonal antibody targeting B cells, which has been used with success in a wide variety of autoimmune diseases. The experience with this drug in patients with inflammatory myopathies (IM), nonetheless, is still limited. We review the literature and highlight several aspects in relation to therapy with rituximab in IM. METHODS: We performed a research in the MEDLINE DATABASE. All cases identified from the literature research and cases diagnosed in our Unit were included in the analysis. RESULTS: We identified 49 patients with IM treated with rituximab in the review of the literature carried out (31 female; 18 male), including our patients. Dermatomyositis (DM) was the most common disorder for which rituximab treatment was administered (69.4%). The other diseases treated included polymyositis (PM) 16.3%, antisynthetase syndrome (AS) 8.2%, one case with anti-SRP-syndrome and other with juvenile dermatomyositis. The median time to diagnosis was 48 (0.75-480) months. Sixty-five per cent (65.3%) of patients presented with skin manifestations, 89.8% with muscle weakness, 7.3% with arthritis, 16.3% with interstitial lung disease, and 7.3% with cardiomyopathy. Seventy-one (71.4%) of the patients received only one course of rituximab, 18.4% two courses, 4.1% three, 2% four and only 4.1% five. We have observed both among our patients and those reported in the literature a high rate of response to rituximab, 75% of our patients and 72.5% of those described in the literature showed a good response. The median time free of symptoms between two courses was 12 (6-19) months. Rituximab was generally well tolerated by all patients, with no serious adverse events. Most of the adverse events reported were mainly infections, particularly respiratory tract infections. CONCLUSIONS: It is our belief that rituximab may be an optimal therapeutic choice for inflammatory myopathies. Nevertheless, there is a need for additional studies in order to assess the optimal regimen of treatment in the different subsets, as well as the initial dose, combination of treatments and re-treatment schedule.

[Necrotizing systemic sarcoidosis with pulmonary and central nervous system involvement]. / Sarcoidosis sistémica necrotizante con afectación pulmonar y del sistema nervioso central.

Sarcoidosis is a multisystemic disease which diagnosis depends on the presence of nonnecrotizing granulomas in the biopsy. However there are variants such as necrotizing sarcoidal granulomas or nodular sarcoidosis which have atypical findings and make difficult the differential diagnosis with other infectious processes. We describe a case of a man who develops granulomas with extensive necrosis in a systemic sarcoidosis that affected the lung and the central nervous system. This finding made us to make the diagnosis of tuberculosis and delay the specific treatment.

Severe Raynaud syndrome induced by adjuvant interferon alfa in metastatic melanoma.

Melanoma is the most lethal form of skin malignancy because of its aggressive behaviour. In advanced disease, interferon alfa can be used as adjuvant therapy. However, this therapy is not free of side effects. We present a case of severe Raynaud syndrome and digital necrosis induced by interferon alfa in a patient with melanoma. Pathogenic mechanisms are discussed.