Assessment of the clinical features of rheumatoid arthritis-related interstitial lung diseases: a retrospective evaluation.

OBJECTIVE: Rheumatoid Arthritis (RA) stands as the most prevalent form of inflammatory arthritis, affecting approximately 1% of the population. Among individuals diagnosed with RA, a notable proportion, ranging from 10% to 40%, also experience Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD). This coexistence of RA and ILD has been identified as a detrimental factor contributing to increased mortality rates. Furthermore, RA-ILD often exhibits an insidious nature, posing challenges in its timely detection and management. Hence, our objective was to conduct a retrospective analysis of the clinical characteristics observed in patients who underwent evaluation for RA-ILD. PATIENTS AND METHODS: A total of 87 patients who were evaluated for RA-ILD within one year were included in the study. This study was conducted retrospectively using a cross-sectional and descriptive approach to analyze the demographic and clinical data of the included patients. RESULTS: Among the 87 patients, eight were diagnosed with RA-ILD, with four being male and four being female. Of the eight patients, two had non-specific interstitial pneumonia, five had usual interstitial pneumonia, and one had nodules consistent with RA. Subpleural fibrosis increased the likelihood of RA-ILD by 6.9 times. In the group with ILD, the residual volume and total capacity were found to be lower compared to the other group. Among the eight patients diagnosed with RA-ILD, five had used methotrexate before the diagnosis. CONCLUSIONS: In order to mitigate the risk of delayed diagnosis of RA-ILD, which can lead to increased mortality and has a subtle onset, it is recommended that patients with RA who possess certain risk factors undergo regular monitoring. It is advisable for RA patients to undergo annual assessments involving carbon monoxide diffusion capacity and spirometry function tests. In cases, where deemed necessary, more advanced investigations such as high-resolution computed tomography should be conducted.

Neutrophil volume distribution width as a new marker in detecting inflammatory bowel disease activation.

INTRODUCTION: We sought to investigate the value of neutrophil volume distribution width in detecting inflammatory bowel disease activation. METHODS: Patients with infection and accompanying inflammatory disease were excluded. All the patients were diagnosed and classified according to Porto criteria and Paris classification, respectively. Physician global assessment, pediatric Crohn's disease and pediatric ulcerative colitis activity indexes and fecal calprotectin were used to define disease activation. RESULTS: A total of 34 pediatric patients with Inflammatory bowel diseases (IBD) and 29 controls were enrolled in the study. Neutrophil volume distribution width (NVDW) was significantly higher in patients with IBD compared to healthy controls (P < 0.001). An increased NVDW level was observed in IBD patients in activation (22.42 ± 2.13) compared to those in remission (19.22 ± 1.63) (P < 0.001). There was no statistically significant difference between IBD patients in remission and healthy controls. The best cutoff of NVDW for prediction of disease activation in Crohn's disease and ulcerative colitis in this series was 20.39 with a sensitivity of 90.9% and a specificity of 75% (AUC: 0.852 CI: 0.698-1.000 P < 0.001) and 19.74 with a sensitivity of 92.9% and a specificity of 90.9% (AUC: 0.961, CI: 0.889-1.000, P < 0.001), respectively. CONCLUSIONS: As a quantitative, objective, and sensitive parameter, we believe that the NVDW has a potential to be an additional test detecting disease activation in IBD.

Relative hypoaldosteronism in a patient with Wolcott-Rallison syndrome.

BACKGROUND: Wolcott-Rallison syndrome is an autosomal recessive, multisystem disorder with onset of diabetes in the neonatal period or early infancy. CASE REPORT: A 9-year-old girl with diabetes and growth failure from 2 months of age presented with ketoacidosis and multiple organ failure. Evaluation for short stature revealed epiphyseal dysplasia. A homozygous mutation in the EIF2AK3 gene confirmed the clinical diagnosis of Wolcott-Rallison syndrome. She was euthyroid. Biochemical evaluation for potential adrenal dysfunction because of persistently elevated serum potassium (range 5.9-6.3 meq/l) and low serum sodium levels (range 128-130 meq/l) 2 weeks after resolution of ketoacidosis yielded normal findings with respect to basal corticotropin (31 pg/ml) and cortisol (18.7 µg/dl) levels. Estimated GFR-Schwartz (36.9 ml/min/1.73 m(2) ) was consistent with stage 3 chronic renal failure. The transtubular potassium gradient was 1.39 (normal value in hyperkalemic states: > 4.1). The plasma aldosterone (upright: 241.3 pmol/l) was within normal ranges, and plasma renin [39 pg/ml (range 5.41-34.53 pg/ml)] was slightly elevated. The patient was diagnosed as having relative hypoaldosteronism and was started on a sodium-rich diet and low potassium. Failure to respond to the dietary intervention prompted a trial of oral fludrocortisone with subsequent normalization of electrolyte levels. CONCLUSIONS: This is the first case report of Wolcott-Rallison syndrome complicated with relative hypoaldosteronism. Further research is needed to probe the causal inference of relative hypoaldosteronism with chronic renal failure in patients with Wolcott-Rallison syndrome.