PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome: A rare cause of childhood neutropenia associated with systemic inflammation and hyperzincemia.

Neutropenia in pediatric patients can be due to a variety of disorders. We describe two patients who underwent extensive evaluation over many years for arthralgias and moderate neutropenia of unclear etiology. Genetic testing identified a pathogenic variant in PSTPIP1 (proline-serine-threonine phosphatase-interacting protein 1) in both patients. Markedly elevated inflammatory markers and zinc levels confirmed the rare diagnosis of PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome, tailoring treatment. Neutropenia is common in patients with PAMI syndrome. Unique mutations seen in PAMI syndrome may account for the specific phenotypic features of this disorder.

An Unusual Case of Fever in Paralytic Ileus.

Still's disease is frequently a condition of exclusion for patients with an unidentified cause of fever. Accompanying symptoms typically include fever, arthralgia, and a transient skin rash. The underlying pathophysiology indicates an autoimmune origin. Diagnosis is primarily clinical, often utilizing the Yamaguchi criteria. The case in question involves a 19-year-old male presenting with high-grade fever and paralytic ileus. The patient received intravenous glucocorticoids and cyclophosphamide, resulting in a rapid clinical improvement. During the follow-up, tofacitinib was initiated based on the clinical response observed.

Investigation of the Effect of Oral Selenium on the Reduction of Clinical Symptoms and Joint Pain in Patients With Rheumatoid Arthritis in the Iranian Population.

Rheumatoid arthritis (RA) is a common inflammatory joint disease. Because inflammation and nitrosative stress play an important role in the pathogenesis of RA, drugs that have antioxidant and anti-inflammatory effects can be effective as adjuvant treatment in these patients. Selenium is a compound that has been shown in recent studies to have anti-inflammatory and antioxidant effects. Therefore, the aim of this study was to investigate the effect of oral selenium on the reduction of clinical symptoms and joint pain in patients with RA. Fifty-one patients with moderate and severe RA were randomly divided into selenium and placebo groups. The first group of patients received selenium at a dose of 200 µg twice a day for 12 weeks along with standard RA interventions and treatments, and the second group received standard treatments of RA along with a placebo. Clinical symptoms were evaluated with standard indicators to evaluate disease activity before and after the intervention in the 12th week. Examination of clinical symptoms at the end of the study showed that in the selenium group and after 12 weeks, a reduction in clinical symptoms and joint pain were observed, which was statistically significant compared with before the study began. Meanwhile, no significant changes were observed in the patients of the placebo group in terms of reducing symptoms and joint pains. A dose of 200 µg of oral selenium twice a day for 12 weeks can significantly reduce clinical symptoms and joint pain in patients with RA.

Acute Onset Rheumatoid Vasculitis With Polyarthritis and Erythema: A Case Report.

We present the case of a woman in her 70s who was diagnosed with rheumatoid vasculitis (RV) after initially presenting with systemic joint pain and erythema. RV, a rare complication of rheumatoid arthritis, involves inflammation of blood vessels, leading to various skin manifestations. The patient's complaints included fever, generalized joint pain, and skin manifestations that initially resembled erythema multiforme. However, a skin biopsy revealed vasculitis, which guided the RV diagnosis. Although rheumatoid arthritis primarily affects the joints, systemic implications such as RV can arise in rare cases. This case underscores the importance of a holistic and meticulous diagnostic approach, especially in older patients, as early detection and treatment are crucial for managing disease progression and associated complications. Collaborative care involving multidisciplinary teams is vital to achieving optimal outcomes in complex cases.

Diagnosis of Rheumatoid Vasculitis From Ischemic Change in Hands: A Case Report.

Rheumatoid arthritis (RA) is a chronic inflammatory disorder with a wide clinical heterogeneity. Among its complications, rheumatoid vasculitis (RV) is notable for its severity and potential to involve multiple organ systems. A particularly serious manifestation of RV is ischemia, which is indicative of advanced vasculitic involvement and a significant risk of tissue damage. This case report describes an 83-year-old male with RA who presented with polyarticular joint pain and hand ischemia. Despite the initial diagnosis of RA exacerbation, worsening systemic symptoms without identifiable infectious causes and hypocomplementemia led to the diagnosis of RV exacerbation. Initial management with steroids showed temporary improvement. However, relapse after dose reduction prompted the administration of rituximab, an anti-cluster-of-differentiate-20 (anti-CD20) monoclonal antibody, which yielded favorable outcomes. This case underscores the importance of clinical vigilance in older patients with RA for signs, such as ischemic hands, emphasizing the pivotal role of early detection and intervention in RV management, particularly in community hospital settings.

Biphasic Zika Illness With Rash and Joint Pain.

During the current Zika virus (ZIKV) outbreak, acute symptomatic ZIKV infection in adults appears to be a mild-to-moderate, self-limited illness. We present a case of ZIKV rash illness that improved and then relapsed without repeat exposure to ZIKV. Clinicians should be alert for relapses in patients with ZIKV infection.

Ultrasound-detected subclinical inflammation was better reflected by the disease activity score (DAS-28) in patients with suspicion of inflammatory arthritis compared to established rheumatoid arthritis.

Limited data are available about the ultrasound (US)-detected inflammatory features in patients with suspicion of inflammatory arthritis (S-IA) vs. established rheumatoid arthritis (RA). Our study aimed to assess if the presence of power Doppler (PD) can be predicted by a combination of clinical, laboratory and US parameters. We conducted a real-life, retrospective cohort study comparing clinical, laboratory and US parameters of 108 patients with established RA and 93 patients with S-IA. We propose a PD signal prediction model based on a beta-binomial distribution for PD variable using a mix of outcome measures. Patients with RA in clinical remission had significantly more active inflammation and erosions on US when compared with patients with S-IA with similar disease scores (p = 0.03 and p = 0.01, respectively); however, RA patients with different disease activity score (DAS-28) scores had similar PD scores (p = 0.058). The PD scores did not correlate with erosions (p = 0.38) or DAS-28 scores (p = 0.28) in RA patients, but they correlated with high disease activity in S-IA patients (p = 0.048). Subclinical inflammation is more common in patients with RA in clinical remission or with low disease activity than in patients with S-IA; therefore, US was more useful in assessing for true remission in RA rather than diagnosing IA in patients with low disease activity scores. This is the first study to propose a PD prediction model integrating several outcome measures in the two different groups of patients. Further research into validating this model can minimise the risk of underdiagnosing subclinical inflammation.

Obesity indices as predictors of pattern of self-reported arthritis among elderly women in Southwestern Nigeria.

OBJECTIVES: This study assessed obesity indices and pattern of arthritis among elderly women in Osun State, Southwestern Nigeria. METHODS: The cross-sectional descriptive study was carried out among 350 elderly women selected by multistage sampling technique. Research instruments used in data collection were semi-structured, interviewer-administered pre-tested questionnaires that were analyzed using the Statistical Package for Social Sciences software version 17.0. RESULTS: The mean age of the participants was 69.1 (±1.4) years. A total of 232 (66.3%) of them had complaints of joint pains (Awoka as expressed in the local language). Pattern of arthritis showed that 172 (74.0%) had recurrent joint pains, while 35 (15.1%) had joint swelling; the knee joint was mostly affected. A total of 97 (28.0%) were overweight while 84 (24.0%) were obese. Obese women were twice more likely to have arthritis when compared to the nonobese (odds ratio: 2.4, 95% confidence interval: 1.8-9.4, p = 0.001). CONCLUSION: There is a need to give high priority to weight reduction strategies targeted at the elderly and the entire population.