OBJECTIVE: Sydenham chorea is an immune-mediated neuropsychiatric condition, and a major criterion for diagnosis of acute rheumatic fever (ARF). Children in remote Northern Australia experience disproportionately high rates of ARF, yet studies looking at the epidemiology, clinical presentation and management of Sydenham chorea are limited in this population.
METHODS: We conducted a retrospective case series from January 2002 to April 2022 of all paediatric patients aged ≤18 years admitted to Royal Darwin Hospital with Sydenham chorea. Cases were identified using the hospital\'s clinical coding system (ICD10). Medical records were reviewed and data on demographics, clinical presentation, investigation results, treatment and outcome were extracted, deidentified and analysed.
RESULTS: One hundred ten presentations of Sydenham chorea occurred between 2002 and 2022, 109 (99%) of these were in First Nations children, with 85% residing in very remote locations. Most commonly, chorea presented as a generalised movement disorder affecting all four limbs (49%). Neuropsychiatric symptoms were reported in 33 (30%), and there was evidence of rheumatic heart disease on echocardiogram in 86 (78%) at presentation. All patients received benzathine penicillin, but there was significant variation in management of chorea, ranging from supportive management, to symptomatic management with anticonvulsants, to immunomodulatory medications including corticosteroids.
CONCLUSIONS: This case series highlights the significant burden of Sydenham chorea among First Nations children living in Northern Australia and demonstrates wide variation in treatment approaches. High-quality clinical trials are required to determine the best treatment for this disabling condition.

Rheumatic heart disease (RHD) is commonly seen in people from developing and low-income countries. More cases are being recorded in developed countries due to migration and globalization. RHD develops in people with a history of rheumatic fever; it is an autoimmune response to group A streptococcal infection due to similarities at the molecular level. Congestive heart failure, arrhythmia, atrial fibrillation, stroke, and infective endocarditis are a few of the many complications associated with RHD. Here we present a case of a 48-year-old male with a past medical history of rheumatic fever at the age of 12 years, who presented to the emergency room (ER) complaining of bilateral ankle swelling, dyspnea on exertion, and palpitations. The patient was tachycardic with a heart rate of 146 beats per minute and tachypneic with a respiratory rate of 22 breaths per minute. On physical exam, there was a harsh systolic and diastolic murmur at the right upper sternal border. A 12-lead electrocardiogram (EKG) revealed atrial flutter with a variable block. Chest X-ray revealed an enlarged cardiac silhouette with a pro-brain natriuretic peptide (proBNP) of 2,772 pg/mL (normal ≤ 125 pg/mL). The patient was stabilized with metoprolol and furosemide and was admitted to the hospital for further investigation. Transthoracic echocardiogram showed left ventricular ejection fraction (LVEF) of 50-55% with severe concentric hypertrophy of the left ventricle with a severely dilated left atrium. Increased thickness of the aortic valve with severe stenosis and a peak gradient of 139 mm Hg and a mean gradient of 82 mm Hg was noted. The valve area was measured to be 0.8 cm2. Transesophageal echocardiogram showed a tri-leaflet aortic valve with commissural fusion of valve cusps with severe leaflet thickening consistent with rheumatic valve disease. The patient underwent tissue aortic valve replacement with a bioprosthetic valve. The pathology report showed extensive fibrosis and calcification of the aortic valve. The patient came in for a follow-up visit 6 months later and expressed feeling better and more active.

UNASSIGNED: Sydenham chorea is thought to be an autoimmune condition that usually develops following a group A beta-hemolytic streptococcal infection.The onset of Sydenham chorea in adults is rare and most of the adult cases usually are secondary to recurrence following childhood illness. Risk factors for chorea recurrence include irregular antibiotic prophylactic use, failure to reach remission within 6 months, and symptom persistence for longer than a year.
UNASSIGNED: A 27-year-old young adult Ethiopian female patient with chronic rheumatic valvular heart disease for the last 8 years experienced repetitive uncontrollable movements of her extremities and torso for three years prior to her current visit. Physical examination was significant for holosystolic murmur at the apical area radiating to the left axilla and choreiform movements apparent on all limbs and trunk. Investigations were significant for mildly raised ESR, echocardiography findings of thickened mitral valve leaflets and severe mitral regurgitation. She was successfully treated with valproic acid and the frequency of penicillin injection was made every 3 weeks with no recurrence for the first 3 months follow-up period.
UNASSIGNED: We believe that this is the first case report of adult onset recurrent Sydenham chorea (SC) from a resource-limited setting. Though Sydenham chorea and its recurrence is rare in adults, it should be considered in adults after ruling out other competing differential diagnoses. Because of the lack of evidence on treatment of such rare cases, individualized mode of therapy is advised. Valproic acid is preferred for symptomatic treatment and more frequent benzathine penicillin G injections, for example every three weeks, may help in the prevention of recurrence of Sydenham chorea.

Rheumatic fever (RF) is a significant public health problem in underdeveloped countries, and its diagnosis is based on modified Jones criteria. However, there are rare manifestations not included in these criteria that can complicate this condition. We present a case report of a 21-year-old Moroccan female with RF revealed by pulmonary involvement. The patient had no known rheumatic fever. She presented with a two-week history of joint pain, severe chest pain, and shortness of breath. On clinical examination, she was febrile with a palpable left knee joint effusion. Laboratory tests indicated elevated levels of inflammation markers and moderate hepatic cytolysis. The thoracic CT scan revealed extensive bilateral alveolar-interstitial parenchymal involvement. The left knee joint puncture showed an inflammatory fluid without germs or microcrystals. Antibiotic therapy with ceftriaxone and gentamycin was ineffective. Echocardiography revealed rheumatic poly valvulopathy with mitral valve narrowing and moderate to severe mitral insufficiency. Streptolysin O antibody levels were high. The diagnosis of RF complicated by rheumatic pneumonia was made. Treatment with amoxicillin and prednisone led to favorable outcomes.

Rheumatoid vasculitis (RV) is a rare but potentially devastating complication of rheumatoid arthritis (RA). It typically occurs in patients with extra-articular manifestations. Here we reported a case of PUK with nodular episcleritis and pulmonary nodules that occurred in the same patient without joint involvement.
A 43-year-old Chinese woman, exhibited a partial crescent-shaped marginal corneal ulcer in the right eye at admission and the ulcer developed rapidly into nearly 360-degree ulcers in both eyes within one week. Nodular episcleritis was observed in the right eye. Conjunctival biopsy revealed vasculitis. Her rheumatoid factor (RF) and anti-cyclic citrullinated protein antibody were positive, while anti-neutrophilic cytoplasmic antibody (c-ANCA) and anti-protease 3 were negative. Pulmonary nodules were found, without joint involvement. The ocular condition did not relieve under the topical and systemic use of corticosteroids, or under other immunosuppressive agents until the infliximab therapy. PUK recurrence was observed after the discontinuation of infliximab.
Rapidly deteriorated PUK with nodular episcleritis and pulmonary nodules occurred in the same patient is a special case of RA without joint involvement. This case reinforces the concept that RV may be the initial sign of RA. Infliximab can be used to prevent further progress of RA-related PUK in some refractory cases.

A previously healthy 8-year-old Native American female presented with right-sided weakness and joint pain for two weeks. Following an initially unremarkable workup including negative brain and spine MRI she was noticed to have chorea and subsequently diagnosed with acute rheumatic fever (ARF). ARF is a group A streptococcus-related disease that most commonly is a sequelae of pharyngitis. The diagnosis of ARF utilizes the Jones criteria which includes heart disease, arthritis, chorea, the characteristic rash of erythema marginatum, and subcutaneous nodules. The most serious consequences of ARF include rheumatic heart disease and chorea. ARF can be treated with a combination of antibiotics and anti-inflammatories like aspirin.

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OBJECTIVE: To explore at the molecular level the phenotype of a patient suffering an autoinflammatory syndrome which was diagnosed as familial cold autoinflammatory syndrome type 2 (FCAS-2). To explore the functions of Nlrp12 in inflammation using mouse models.
METHODS: Whole exome sequencing and Nlrp12 targeted resequencing were performed on DNA isolated from the patient and her family members. In vivo and ex vivo models of inflammation (urate crystals-dependent acute joint inflammation and urate crystals-induced peritonitis) were analysed in Nlrp12-deficient and Nlrp12-competent mice.
RESULTS: A rare missense NLRP12 variant (c.857C>T, p.P286L) was identified in the patient and her healthy relatives. Nlrp12-deficient mice exhibit reduced systemic inflammation and neutrophilic infiltration.
CONCLUSIONS: Nlrp12 mediates proinflammatory functions in mice. In humans, the identification of Nlrp12 variants must be cautiously interpreted depending on clinical and paraclinical data to diagnose FCAS-2.

BACKGROUND: Rheumatic diseases and vaginal infections both increase the risk of preterm birth. It is unclear whether pregnant women with rheumatic disease are more likely to experience vaginal infections, which might potentially accumulate modifiable risk factors.
OBJECTIVE: In this study, we sought to evaluate the vaginal microbiota of pregnant women with inflammatory rheumatic and inflammatory bowel disease.
METHODS: A total of 539 asymptomatic women with singleton pregnancy were routinely screened for an abnormal vaginal microbiota between 10 + 0 and 16 + 0 gestational weeks. Vaginal smears were Gram-stained and microscopically analysed. Those with inflammatory diseases (with or without immunomodulatory therapy) were assigned to the case group and matched in a 1:3 ratio to healthy pregnant controls.
RESULTS: Overall, an abnormal vaginal microbiota occurred more frequently among women of the case group, compared with those of the control group (33.8% vs 15.6%; 95% CI: 1.78-4.27, p < .001). In particular, Candida colonisation (22.3% vs 9.2%; 95% CI: 1.69-4.75, p < .001), but also bacterial vaginosis (14.9% vs 7.2%; 95% CI: 1.25-4.1, p = .006), occurred more often in the case than in the control group. No significant difference was found with regard to the occurrence of an abnormal vaginal microbiota between subgroups with and without immunomodulatory treatment (37.0% vs 27.1%; 95% CI: 0.29-1.35, p = .232).
CONCLUSIONS: Pregnant women with inflammatory rheumatic and inflammatory bowel disease are at risk for bacterial vaginosis and Candida colonisation, which might pose a risk for preterm birth. Prospective studies are needed to further evaluate the influence of autoimmune conditions and immunosuppressive therapy on the vaginal microbiota.

BACKGROUND: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF.
METHODS: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs.
CONCLUSIONS: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.