Clinical Rheumatology

The case histories of 3 elderly patients who developed haemarthrosis of osteoarthritic joints with subsequent infection with staphylococcus aureus, are described. Trauma to the affected joints was a predisposing factor in 2 patients and, while only one patient developed clinical signs of sepsis, all had marked elevation of the erythrocyte sedimentation rate. Although suspicion of joint sepsis was obscured by the presence of a coexistent haemarthrosis, routine culture of joint aspirates showed infection with staphylococcus aureus and all patients recovered well with antibacterial therapy.

The objective of this study is to assess the reliability of clinical examination in patients with erosive osteoarthritis (EOA). Eighteen patients with EOA underwent clinical examination for joint tenderness, bony swelling, and inflammation by two independent, blinded assessors. All patients were also examined by ultrasound (US) by an independent radiologist. The inter-observer agreement was moderate for bony swelling and joint tenderness and fair for joint inflammation (κ = 0.513, 0.448, and 0.402, respectively). US detected significantly more inflamed joints than clinical examination. The sensitivity and specificity of clinical examination for joint inflammation were 0.12 and 0.95, respectively. Clinical joint counts for bony swelling, tenderness, and inflammation all correlated with functional status, assessed by the functional index for hand osteoarthritis (FIHOA), whereas US joint counts for joint inflammation did not correlate with the FIHOA. No correlation was found between any clinical or US joint count and visual analog scale for pain. US detects more joints with inflammation than clinical examination in patients with EOA. US can supplement the clinical examination of patients with EOA, as US-detected subclinical joint inflammation might accelerate joint damage and thus functional impairment.

Transfusion-related acute lung injury (TRALI), defined as the onset of acute respiratory distress after blood transfusion, is a rare complication which is a leading cause of transfusion related-mortality. In this retrospective study, we report the French nationwide experience of intravenous immunoglobulin (IVIG)-related TRALI, with a literature review and analysis of management and outcome of this rare condition. With the pharmacovigilance services, we conducted a retrospective multicenter study in the French network of intensive care units with TRALI concomitant to IVIG use and pooled with data from a literature review. Overall, 17 cases have been included in this case-series, our case report, seven personal cases and nine cases from the literature review. The median age was 55 years [2–79] with 10/17 (59%) male subjects. The underlying diseases motivating IVIG infusion were neurologic diseases in 35% of cases (Guillain Barre syndrome = 2, peripheral neuropathy = 2, neurolupus = 1, myasthenia = 1), multiple myeloma with hypogammaglobulinemia (n = 2; 12%), primary hypogammaglobulinemia (n = 2; 12%), autoimmune cytopenias (n = 2; 12%), graft versus host cutaneous disease after allogeneic hematopoietic stem cell transplantation for acute myeloid leukaemia (n = 1), anti-HLA antibodies after lung transplant (n = 1), cancer-associated thrombotic thrombocytopenic purpura-haemolytic uremic syndrome (n = 1), Kawasaki disease (n = 1) and in experimental essay (n = 1). TRALI symptoms begin either after the start or during the infusion (n = 7; 41%), or after the infusion (n = 10; 59%, 10 min to 24 h). Besides respiratory distress, it was also noted shock (33%), fever (18 %), cough (18%), nausea/vomiting (18 %), chills (12%) and agitation (12%). The X-ray showed mainly bilateral alveolar opacities (n = 15; 88%). Mechanical ventilation was needed in nine cases (53%), with median 1-day duration [1–4]. Four patients (24%) died during hospitalisation in the intensive care unit. Given the increasing use of intravenous immunoglobulins, TRALI must now be discussed in cases of respiratory distress occurring during or immediately following the infusion even if this side effect remains rare.

A long-term follow-up of 119 patients with the descriptive diagnosis of B27-positive oligoarthritis showed that after a time between months to several years about half of the patients with this condition develop a definite disease of the seronegative spondarthritis group, especially ankylosing spondylitis. Another great part of the patients go into a complete and presumably persistent remission of their arthritis. After a follow-up time of 8 to 12 years, about 10% present a recurrent B27-associated oligoarthritis with some special clinical features. It is to be discussed whether this condition is a separate disease entity or still an abortive form of a well-known disease of the seronegative spondarthritis group.

Sarcopenia is characterized by a decline in muscle strength, muscle mass, and physical performance. Persistent inflammation may lead to muscle wasting. This research aims to determine the prevalence and associated factors of sarcopenia in axial spondyloarthritis (ax-SpA). A cross-sectional study of 104 ax-SpA patients who met the 2009 ASAS criteria was conducted at Phramongkutklao Hospital between January 2020 and February 2021. Sarcopenia-related factors and disease characteristics were recorded. Sarcopenia was diagnosed according to the Asian Working Group for Sarcopenia 2019. Appendicular skeletal muscle mass was measured by dual-energy X-ray absorptiometry. Strength, ambulation, rising from a chair, stair climbing and history of falling (SARC-F) was evaluated. Muscle strength was measured by hand grip strength and chair stand time. Physical performance was assessed by 6-m walk and time up and go tests. Logistic regression was performed to identify factors associated with sarcopenia. Most patients were male (74%), with a mean (standard deviation, SD) age and disease duration of 42.6 (12.2) and 8.3 (8.5) years, respectively. The mean BMI (SD) was 23.8 (4.4). The prevalence of sarcopenia was 22.1%. There were no differences in disease activity (BASDAI and ASDAS) between ax-SpA patients with and without sarcopenia. Age, low BMI, and BASFI score were independently associated with sarcopenia, with adjusted odds ratios and 95% confidence intervals of 1.08 (1.01–1.16), 0.39 (0.25–0.63), and 1.41 (1.07–1.86), respectively. Sarcopenia is common in ax-SpA patients and is independently associated with older age, low BMI, and high BASFI score but not disease activity.

With galectin-3 playing an important role in inflammatory responses, elevated galectin-3 levels have been shown in patients with autoimmune diseases. However, there are limited data regarding galectin-3 expression in patients with autoinflammatory diseases such as adult-onset Still’s disease (AOSD). This study aimed to investigate the extracellular galectin-3 expression and examine its association with activity parameters and disease outcome in AOSD patients. Plasma levels of galectin-3 and inflammasome downstream cytokines including interleukin (IL)-1β and IL-18 were determined by ELISA in 42 active AOSD patients and 20 healthy controls (HC). The protein levels of galectin-3 and cytokines were determined using immunoblotting. Plasma levels of galectin-3 and inflammasome downstream cytokines including IL-1β and IL-18 were significantly higher in AOSD patients (median 5.02 ng/ml, interquartile range [IQR] 3.12–7.88 ng/ml; 3.42 pg/ml, IQR 1.48–6.70 pg/ml; and 5758 pg/ml, IQR 859-11,895 pg/ml, respectively) compared with HC (1.86 ng/ml, IQR 1.09–2.89 ng/ml; 0.99 pg/ml, IQR 0.62–1.35 pg/ml; and 129 pg/ml, IQR 71-155 pg/ml, respectively, all p < 0.001). Plasma galectin-3 levels were positively correlated with clinical activity scores, inflammatory parameters values, and the levels of IL-1β and IL-18 in AOSD patients. AOSD patients with systemic pattern had significantly higher galectin-3 levels (median 6.08 ng/ml, IQR 4.01–9.54 ng/ml) compared with those with chronic articular pattern (3.56 ng/ml, IQR 3.04–4.98 ng/ml, p < 0.05). After 6-month therapy, galectin-3 levels significantly declined, paralleling the decreases in clinical activity scores and plasma levels of IL-1β and IL-18. Elevated galectin-3 levels and their positive correlation with disease activity scores, inflammatory parameter, and inflammasome downstream cytokines suggest the involvement of galectin-3 in AOSD pathogenesis.

Several studies revealed the epidemiology of Kawasaki disease-related hospitalizations among children in the USA and other countries. However, disparities of developing coronary artery aneurysms by race/ethnicity, patient socioeconomic status, and geographic locations remain unknown in the USA. Hospital discharge record data of patients with Kawasaki disease aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kid’s Inpatient Database. The data were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Multivariable logistic regression was conducted to ascertain the factors associated with the development of coronary artery aneurysms. Total annual hospitalization rates of Kawasaki disease showed a decreasing trend, ranging from 6.54 per 100,000 children in 2003 to 6.11 per 100,000 children in 2012 (p < 0.001). The proportions of coronary artery aneurysms among patients with Kawasaki disease ranged from 2.25 to 3.20%. Factor associated with the development of coronary artery aneurysms was hospitals in West (OR 2.15, 95% CI 1.42–3.26). Race/ethnicity, health insurance status, and household income were not associated with the development of coronary artery aneurysms. Total hospitalization rates of Kawasaki disease showed a decreasing trend. Children admitted to hospitals in West region were more likely to develop coronary artery aneurysms.

Many autoimmune diseases have been reported to be associated with malignancy. Mixed connective tissue disease (MCTD), however, has rarely been associated with malignancy. Thymoma is one of the neoplasms often reported to be related to various immunological disorders. Among the types of thymoma defined by WHO, malignant thymoma (thymoma type C) is the one least reported to be associated with autoimmune disease. Here, we report a case of malignant thymoma with concurrent MCTD, which manifested with acrosclerosis, Raynaud’s phenomenon, arthritis (synovitis), and a high titer of anti-ribonucleoprotein antibody.