Cerebellar degeneration in primary Sjögren syndrome: a case report

Background Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet’s disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome

Arthritis as an initial presentation of malignancy: two case reports

Abstract Background: Arthritis is rarely reported as a paraneoplastic manifestation of occult malignancy. We report herein two cases of paraneoplastic arthritis due to occult malignancy. Case 1: The patient was a 65-year-old woman of asian descent who was a former smoker with a history of spine surgery performed for L4/L5 degenerative disc disease. She presented with a 1-month history of oligoarthritis afecting both ankle joints and early morning stifness of about 3 hours. Laboratory tests were positive for antinuclear antibody at a titer of 1:320 (speckled) but negative for rheumatoid factor. She was treated for seronegative spondyloarthritis and started on prednisolone without much improvement. A routine chest radiograph incidentally revealed a right lung mass which was found to be adenocarcinoma of the lung. She was treated with geftinib and her arthritis resolved. Case 2: The patient was a 64-year-old woman of asign descent, nonsmoker, who presented with a chief complaint of asymmetrical polyarthritis involving her right wrist, second and third metacarpophalangeal joints, and ifrst to fifth proximal interphalangeal joints. She was treated for seronegative rheumatoid arthritis (RA) and started on sulfasalazine, with poor clinical response. Six months later, she developed abdominal pain which was diagnosed as ovarian carcinoma by laparotomy. Her arthritis resolved following treatment of her malignancy with chemotherapy. Conclusion: In summary, paraneoplastic arthritis usually presents in an atypical manner and responds poorly to disease-modifying antirheumatic drugs. Accordingly, we recommend screening for occult malignancy in patients presenting with atypical arthritis.

Clinical features of patients with rheumatic diseases and COVID-19 infection in Sarawak, Malaysia

We read with great interest the article by Ye et al1 describing the clinical features and outcomes of patients with rheumatic diseases and COVID-19 in Wuhan, China. It concluded that length of hospital stay and mortality were similar between patients with rheumatic diseases and non-rheumatic groups, while respiratory failure was more common in patients with rheumatic diseases infected with COVID-19. D’Silva et al2 and Zhao et al3 subsequently highlighted the differences of clinical severity and outcomes in their respective cohorts of patients with rheumatic diseases and COVID-19. Fredi et al4 presented data from northern Italy which supported an association of elderly age and the presence of comorbidities with a poor outcome of COVID-19 infection, rather than the type of rheumatic disease or background medications. The Global Rheumatology Alliance5has recently published data of characteristics associated with hospitalisation for COVID-19 among patients with rheumatic diseases. We would like to share the clinical course of COVID-19 among patients with rheumatic diseases in Sarawak

Acute pancreatitis as an initial presentation of SLE: a case report

Background: SLE is a complex multi- systemic autoimmune disease capable of affecting any organ system with varying presentations. Abdominal pain is a common manifestation of SLE and is reported to occur in about 8% to 40% of patients with SLE. The causes of abdominal pain are varied and require accurate assessment. Acute Pancreatitis is a cause of abdominal pain and is a rare initial SLE presentation. It is characterized by abdominal pain and raised serum amylase levels. We would like to report a patient diagnosed with SLE following an initial presentation of Acute Pancreatitis, which was complicated with a pancreatic pseudocyst. Case Report: The patient is an 18- year- old lady with no previous known medical illness who presented with a one day history of acute central abdominal pain radiating to the back. This was preceded by a two week history of fever and non- productive cough. Further history revealed that she had alopecia, malar rash and painless oral ulcers one year before this presentation but did not seek medical attention. Physical examination revealed a tender and distended abdomen. Blood investigations showed leukopenia, elevated amylase with a positive Anti- nuclear antibody, and coombs test. She was admitted to the ICU and treated with intravenous cyclophosphamide, methylprednisolone, immunoglobulin, and antibiotics. During her follow- up a month after discharge, she was found to have recurrent abdominal pain and distension. A repeated CT Abdomen showed a pancreatic pseudocyst, and she underwent an endoscopic cystogastrostomy. Currently, she is on regular follow- up and is currently maintained on Hydroxychloroquine, Azathioprine, and Prednisolone. Conclusion: In summary, Acute Pancreatitis is a relatively rare initial manifestation of SLE. The causes of Acute Pancreatitis are varied, and it is essential to recognize and differentiate Acute Pancreatitis due to active SLE from other causes as it may affect treatment decisions and subsequent mortality outcomes

IgG4- related disease, a case series from Sarawak Malaysia

Background: IgG4- related disease (IgG4- RD) is a novel entity of disease first recognised in 2003. It has wide spectrum of clinical manifestations, virtually affecting any organ. It’s epidemiology remain poorly described and treatment guideline still lacking. Methods: We describe 7 IgG4RD cases diagnosed and treated in 2 hospitals with rheumatologist in Sarawak from 2015 till 2020. Results: 4 males and 3 females were reported with age during diagnosis ranging from 34 to 74 years old. 4 patients had tumefactive lesions (lacrimal gland and salivary gland swelling), 2 had obstructive symptoms (obstructive uropathy due to retroperitoneal fibrosis, obstructive jaundice secondary to autoimmune pancreatitis) and 1 had renal failure and proteinuria as presenting symptoms. Time taken from onset of symptoms to diagnosis range from 8 months to 23 years. In all cases, histopathological findings were the prompt towards diagnosis of IgG4- RD. All patients fulfilled the ACR- EULAR classification criteria whereas only 3 met the 2010 JCR comprehensive diagnostic criteria, due to lack of IgG4 level in others. All patients showed rapid response to steroid therapy, complete resolution of salivary gland swelling were seen in 3 months, stent removal in those with obstructive symptoms were achieved in 6 months whereas resolution of proteinuria was seen in 2 months. All remained in remission currently with or without treatment. Initial dose of prednisolone used ranged from 0.2 to 0.6mg/kg/day, all with good effect. Conclusion: IgG4- RD is indolent but often highly destructive. It is treatable and if diagnosed early, damage is potentially reversible. In terms of diagnosis, we feel that the ACR- EULAR classification criteria is more practical compared to the JCR criteria in places where resources are limited and laboratory testing of serum IgG4 level is not available. Good awareness and high index of suspicion among clinicians, radiologists and pathologists are thus essential in timely diagnosis and prompt treatment of IgG4- RD

Language choice patterns of multilingual speakers in Sebuyau

This paper reports preliminary results of an on-going research project undertaken to study the language choice patterns of multilingual communities in Sebuyau, a small coastal town situated at the border between Sri Aman and Simunjan. The research is part of a bigger project which examines language contact and multilingual communities in the south-western region of Sarawak i.e. in the district of Lundu, Sebuyau and Simunjan. In language contact situations, it is not languages that come into contact but speakers of the languages. Therefore, language attitude (e.g. perceptions towards each other’s language and own language), and the link between language and identity are crucial factors that will influence language choice (Appel & Musyken, 1987; Fishman, 1977; Grosjean, 1982; Norazuna, 2009; c.f. Sercombe, 2000). Data for this paper were collected through in-depth interview with the help of a questionnaire. In addition to the questionnaire, general observation of language choice behaviour of the Sebuyau in various social situations was conducted. The results suggest, among others, that the younger generation Iban are accommodating to the language of their Malay interlocutors in interaction. This change in norms of language use in Malay-Iban interaction may be also reflect attitudes towards languages

Fatal gastrointestinal manifestations of SLE: case reports

Background: SLE- related gastrointestinal involvement such as gastrointestinal vasculitis and thrombosis are clinically important, as it could progress to life- threatening outcome if not treated promptly. We describe 2 patients who were admitted to our hospital with fatal gastrointestinal manifestations of SLE. Method: Case Report. Results: Case 1: The patient was a 24- year- old Asian woman who was diagnosed with SLE in 2014 and class IV lupus nephritis in 2018. She presented with hypovolemic shock secondary to massive hematochezia. CT abdomen and angiography revealed multiple bleeding sites in jejunum and mesenteric vasculitis changes of the bowels. Gastroduodenoscopy and CT angioembolization of the mesenteric arteries were performed to stop the bleeding, and IV Methylprednisolone, IV Cyclophosphamide, IV Rituximab were administered. Eventually she succumbed to Transfusion- Related Acute Lung Injury (TRALI) from multiple blood transfusions. Case 2: The patient was a 61- year- old male smoker who was diagnosed with SLE with lupus nephritis in April 2020. He however refused renal biopsy or cyclophosphamide. He presented a few months later with fever and colicky abdominal pain for 2 days. A diagnosis of Superior Mesenteric Artery (SMA) Thrombosis with Bowel Ischemia was made, which was likely due to Catastrophic Anti- Phospholipid Syndrome (CAPS) with SLE, based on CT abdomen findings of SMA thrombosis, bilateral common femoral vein thrombosis and multiple splenic infarcts. Heparin infusion was initiated, and he was administered IV Methylprednisolone and Intravenous Immunoglobulin (IVIg). He developed Disseminated Intravascular Coagulation (DIVC) subsequently and succumbed to his illness at day 2 of admission. Conclusion: In summary, early diagnosis with prompt and adequate treatment of gastrointestinal manifestation of SLE is essential to avoid serious complications like haemorrhage or perforation with a high mortality rate