JAK inhibitors in refractory dermatomyositis: A case series.

This retrospective cohort study assessed the efficacy and safety of Janus kinase (JAK) inhibitors, tofacitinib and baricitinib, in 14 patients with refractory dermatomyositis (DM), a multisystemic autoimmune disorder with limited therapeutic options. Results demonstrated a significant median decrease of 21 points and a 76% reduction in the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) scores, along with a complete resolution of muscular symptoms in 64% of the patients. JAK inhibitors were effective in managing refractory DM across various subtypes with mild and manageable adverse events.

Low serum osteocalcin levels are associated with diabetes mellitus in glucocorticoid treated patients.

Bone turnover markers are decreased in GC-treated subjects with DM. Decreased OC levels in GC-treated patients were associated with an increased risk of DM. These results suggest the involvement of OC in glucose homeostasis regulation in DM. INTRODUCTION: Osteocalcin (OC) is involved in the regulation of glucose homeostasis. Glucocorticoid (GC) treatment is associated with impaired osteoblast function, decreased OC levels, and the development and/or worsening of pre-existing diabetes mellitus (DM). Whether decreased OC levels in GC-treated subjects contribute to DM is not well known. The aim of this study was to analyse whether OC levels in GC-treated patients are associated with the presence of DM. METHODS: One hundred twenty-seven patients (aged 61.5 ± 17.9 years) on GC treatment were included. GC dose, treatment duration, presence of DM and bone formation (OC, bone ALP, PINP) and resorption markers (urinary NTX, serum CTX) were analysed. The cut-offs of each bone turnover marker (BTM) for the presence of DM were evaluated and optimised with the Youden index and included in the logistic regression analysis. RESULTS: Among the patients, 17.3% presented DM. No differences were observed in GC dose or duration or the presence of fractures. Diabetics showed lower levels of OC (7.57 ± 1.01 vs. 11.56 ± 1; p < 0.001), PINP (21.48 ± 1.01 vs. 28.39 ± 1; p = 0.0048), NTX (24.91 ± 1.01 vs. 31.7 ± 1; p = 0.036) and CTX (0.2 ± 1.01 vs. 0.3 ± 1; p = 0.0016). The discriminating BTM cut-offs for DM presence were < 9.25 ng/mL for OC, < 24 ng/mL for PINP, < 27.5 nMol/mM for NTX and < 0.25 ng/mL for CTX. In a multivariate logistic regression model adjusted for GC dose, BMI, age and the above four BTMs, only OC remained independently associated with DM presence. Thus, in a model adjusted for GC dose, BMI and age, OC was significantly associated with DM (OR: 6.1; 95%CI 1.87-19.89; p = 0.001). CONCLUSION: Decreased OC levels in GC-treated patients are associated with increased odds of DM, and only OC was independently associated with DM in a model including four BTMs.

Renal tubular acidosis type IV as a complication of lupus nephritis.

Renal tubular acidosis (RTA) is a rare complication of renal involvement of systemic lupus erythematosus (SLE). We describe a 24-year-old male with type IV lupus nephropathy as a presenting manifestation of SLE. He presented with improvement of renal function following induction therapy with three pulses of methylprednisolone and 500 mg biweekly pulses of cyclophosphamide. However, a week after the first pulse of cyclophosphamide, the patient presented with a significant increase in legs edema and severe hyperkalemia. Type IV RTA associated with hyporeninemic hypoaldosteronism was suspected in the presence of metabolic acidosis with a normal anion gap, severe hyperkalemia without worsening renal function, and urinary pH of 5. RTA was confirmed with a transtubular potassium concentration gradient of 2 and low levels of plasma aldosterone, renin, angiotensin II, and cortisol. Intravenous bicarbonate, high-dose furosemide, and fludrocortisone were administered with normalization of potassium levels and renal function.

Diagnosis and classification of granulomatous myositis.

The term granulomatous myositis is applied to a myopathic syndrome associated with non-specific epithelioid granulomas in striated muscle. This rare entity is most frequently related to sarcoidosis, but other uncommon causes have been reported, including an idiopathic form only after systemic disorders known to cause similar myopathological abnormalities have been excluded. Symmetrical proximal or distal muscle weakness is the rule in the clinical presentation, sometimes associated with dysphagia. Although the clinical profile together with electromyography (EMG) studies may be useful, definite diagnosis requires pathological examination. Systemic glucocorticoids are the treatment of choice, but the clinical outcome is not always satisfactory.

[Improving the care of critical patient family members: Agreed on strategies]. / Mejorando el cuidado a los familiares del paciente crítico: estrategias consensuadas.

INTRODUCTION: Within the context of participatory action research (PAR), a 4-stage process was established with the general aim of promoting improvements in the care offered to families of patients in the Intensive Care Unit (ICU). The 4 stages consisted of a situational diagnosis, proposals for change, the design and implementation of the proposals, and an evaluation. This paper presents the first 2 stages. OBJECTIVES: To define the attention given to families of patients in the Intensive Care Unit. To reach a consensus on areas for action/intervention in the unit. MATERIALS AND METHOD: A qualitative methodology. DESIGN: PAR. Data-collection technique: 4 focus groups made up of 10 professionals, and consensus with support groups made up of 30 members. Content analysis was performed. The theoretical saturation point was reached. RESULTS: Two documents were drawn up: 1) A situational diagnosis, describing the current situation of the attention given to families and 2) 10 proposals for change, 5 of which were given priority. They were: a session to familiarize professionals with evidence concerning families of patients in the ICU and the handing out of informative leaflets, the improvement of (nurse-family and intra-team) informative procedures, more privacy for patients and a reduction in environmental noise, a training plan for professionals, and change in the visiting policy. CONCLUSION: By using consensus-based methodologies, strategies for change can be prioritized and designed, adapted to the context in which they will be applied.

Antifungal activity of some Cuban Zanthoxylum species.

Ethanolic extracts of the trunk bark of Zanthoxylum fagara, Z. elephantiasis and Z. martinicense showed activity against different species of fungi. No antibacterial activity was detected.