Clinical features and outcomes from the Singapore Sjögren's syndrome study.

OBJECTIVE: To study the clinical features, treatment and outcomes of primary Sjögren's Syndrome (pSS) in a Singapore cohort from an outpatient rheumatology clinic. METHODS: Computerised Physician Order entry records of patients who fulfilled the 2016 ACR-EULAR classification criteria for pSS between 1993 and 2013 were retrospectively analysed. RESULTS: There were 102 patients, of which 96 (94.1%) were females, and 91 (89.2%) Chinese. Mean age at diagnosis was 49.3 ± 11.8 years, mean disease duration was 9.0 ± 4.6 years. The most common manifestations were keratoconjunctivitis sicca (99.0%), xerostomia (96.1%), arthralgia/arthritis (56.9%). Exocrine glandular enlargement comprised parotidomegaly (28, 27.5%), with concurrent submandibular and lacrimal gland enlargement in one. The nervous system (15.7%) was the most commonly affected internal organ, with peripheral nervous system (peripheral neuropathy, mononeuritis multiplex) involvement more common than central. Hydroxychloroquine was most frequently used (88.2%), followed by methotrexate (7.8%) and azathioprine (6.9%). Pulsed intravenous (IV) methylprednisolone 500 mg/day for 3 days was used in 5 patients followed by oral (4) or IV cyclophosphamide (1) for cardiomyopathy and interstitial lung disease (1), and neurological involvement (4). These comprised neuromyelitis optica, transverse myelopathy, cranial neuropathy, mononeuritis multiplex and/or peripheral neuropathy alone or in combination. Intravenous immunoglobulins (2.0%) was used for sensory neuropathy and mononeuritis multiplex; rituximab (1.0%) in 1 patient for treatment of non-Hodgkin's B-cell lymphoma. There were no deaths. CONCLUSION: Musculoskeletal manifestations were common, with the nervous system (peripheral more than central) the most common internal organ involved. Lymphoma was uncommon despite up to one-third of the cohort developing glandular enlargement.

Anaesthesia of a parturient with uncorrected pentalogy of Fallot undergoing caesarean section and postpartum sterilisation.

Pentalogy of Fallot is a rare congenital cyanotic heart disease; few patients with uncorrected disease survive to childbearing age. Cardiovascular changes during pregnancy and delivery can lead to haemodynamic instability, while anaesthesia can cause right-to-left shunting and worsen hypoxaemia.We present the learning points from the anaesthetic management of an obstetric patient with uncorrected pentalogy of Fallot. We describe the successful application of general anaesthesia, choice of transoesophageal echocardiography for real-time haemodynamic monitoring and management, and the comprehensive multidisciplinary care of this high cardiovascular risk obstetric patient perioperatively. We also review the literature and discuss the anaesthetic management of patients with pentalogy of Fallot going for caesarean section.

An Unusual Case of Cerebral Arteriovenous Malformation in Pregnancy.

We present a case of a woman at 31 weeks and 3 days of gestation, who developed a sudden and severe headache and loss of vision in her left eye. Magnetic resonance imaging (MRI) of the brain revealed a subarachnoid bleed secondary to a right parieto-occipital arteriovenous malformation (AVM). She was conservatively managed and subsequently transferred to our institution for multidisciplinary care. The patient underwent a cesarean section at 34 weeks and 5 days of gestation followed by gamma knife surgery 6 days after. Cerebral AVMs, although relatively rare, have the propensity to cause potentially fatal outcomes. Neurological symptoms in a pregnant woman warrant investigations for early diagnosis and management, due to its associated morbidity and mortality. The management of cerebral AVMs in pregnancy is decided after weighing the benefits of treatment against the risk of bleeding. A multidisciplinary approach should be adopted due to the complexity of the condition.