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

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

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

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.

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

Mutation signatures implicate aristolochic acid in bladder cancer development

10.1186/s13073-015-0161-3Genome Medicine71Article number 3

Ventricular tachycardia storm as predominant cardiac manifestation of lupus myocarditis

SUMMARY Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease that can affect any part of the heart, causing arrhythmias on top of other cardiac manifestations. Malignant ventricular tachyarrhythmias are rare manifestations of SLE. Our case is the first one reported in the literature of an SLE patient with multi-organ involvement who subsequently presented with ventricular tachycardia (VT) storm as a cardiac manifestation. This case also demonstrates the use of Stellate ganglion block to treat intractable VT storm when chemical and electrical cardioversions failed, while waiting for immunosuppressive drugs to take effect. Timely treatment resulted in a good outcome for our patient

Multivariate visualization of the global COVID-19 pandemic: A comparison of 161 countries.

BackgroundThe aim of the study was to visualize the global spread of the COVID-19 pandemic over the first 90 days, through the principal component analysis approach of dimensionality reduction.MethodsThis study used data from the Global COVID-19 Index provided by PEMANDU Associates. The sample, representing 161 countries, comprised the number of confirmed cases, deaths, stringency indices, population density and GNI per capita (USD). Correlation matrices were computed to reveal the association between the variables at three time points: day-30, day-60 and day-90. Three separate principal component analyses were computed for similar time points, and several standardized plots were produced.ResultsConfirmed cases and deaths due to COVID-19 showed positive but weak correlation with stringency and GNI per capita. Through principal component analysis, the first two principal components captured close to 70% of the variance of the data. The first component can be viewed as the severity of the COVID-19 surge in countries, whereas the second component largely corresponded to population density, followed by GNI per capita of countries. Multivariate visualization of the two dominating principal components provided a standardized comparison of the situation in the161 countries, performed on day-30, day-60 and day-90 since the first confirmed cases in countries worldwide.ConclusionVisualization of the global spread of COVID-19 showed the unequal severity of the pandemic across continents and over time. Distinct patterns in clusters of countries, which separated many European countries from those in Africa, suggested a contrast in terms of stringency measures and wealth of a country. The African continent appeared to fare better in terms of the COVID-19 pandemic and the burden of mortality in the first 90 days. A noticeable worsening trend was observed in several countries in the same relative time frame of the disease's first 90 days, especially in the United States of America