Neuropsychiatric SLE (NPSLE) comprises the neurologic and psychiatric syndromes observed in patients with SLE after exclusion of other causes. The diagnosis of NPSLE is challenging due to diverse clinical manifestations and absence of laboratory or radiologic biomarkers.

We present the case of a patient with SLE with a chronic isolated seizure and was successfully managed with antiepileptic medication and high-dose corticosteroids.

Seizures may occur as an isolated manifestation of an SLE flare. Ischemic and inflammatory causes of seizure may coexist in active lupus and both should be considered in managing patients. A prompt and holistic workup to rule out metabolic, infectious, and structural neural causes and lupus disease activity of seizures is prudent for patients with SLE.

BACKGROUND

Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by inflammatory arthritis and extra-articular involvement. Comorbidities are highly prevalent in patients with RA, in particular cardiovascular disease (CVD), which is responsible for over 50% of premature deaths. This study aimed to describe cardiovascular diseases and their risk factors among patients with rheumatoid arthritis in the Philippine General Hospital (PGH).

To describe cardiovascular (CV) diseases and their risk factors among patients with rheumatoid arthritis.

A retrospective descriptive cross-sectional study was done in the University of the Philippines – Philippine General Hospital (UP-PGH) inpatient and outpatient services. The study included patients 18 years old and above diagnosed with RA and fulfills the 1987 American College of Rheumatology or 2010 American College of Rheumatology-European League Against Rheumatism (ACR/EULAR) classification criteria with no overlap features with other autoimmune connective tissue diseases and with complete records of the information required for the study from January 2019-December 2022. The primary outcomes of interest were the prevalence of CV diseases and CV risk factors. Descriptive statistics were used to summarize the data.

There were 123 patients in the study, 93.4% outpatients, and 95.1% females, with a mean age and disease duration of 51.3 and 9.8 years, respectively. Disease activity was moderate in 35% and high in 9.7%, based on disease activity score (DAS 28) or clinical disease activity index (CDAI) scores. Methotrexate (54%) was the most commonly used conventional synthetic disease-modifying antirheumatic drug (csDMARD). Glucocorticoid use was observed in 51.2%. None of the patients were receiving a biologic DMARD. There were 24 (19.5%) patients with CV diseases, namely myocardial infarction, heart failure, and stroke. There were 87 (70%) patients with at least one CV risk factor and 62 (50.4%) with multiple risk factors. The risk factors identified were: dyslipidemia (43.1%), hypertension (40.7%), elevated body mass index (35.7%), and diabetes mellitus (15.4%). There were f ive deaths in the hospitalized patients (4%), one due to a myocardial infarction.

The majority (70%) in our cohort had at least one CV risk factor, 19.5% had an identified CV disease, and one died from a myocardial infarction. Dyslipidemia was the most common CV risk factor. The high proportion of patients with CV disease and CV risk factors highlights the need to add the screening and management of CV diseases and risk factors as a priority among patients with rheumatoid arthritis.

Objective: To determine the one-year outcomes of newly-diagnosed patients with systemic lupus erythematosus (SLE) in a tertiary government hospital in Manila, Philippines. Methods: After ethics approval, we reviewed the medical records of a cohort of 44 newly-diagnosed SLE patients at 6- and 12-months post-diagnosis in 2018-2019. The outcomes of interest were: modified lupus low disease activity state as defined (mLLDAS), remission, hospitalization, 30-day readmission, organ damage, and mortality. Results: The patients were predominantly young females (mean age of 29 ± 9.9 years). There was an average interval period of six months between onset of symptoms and diagnosis (6.4 ± 10.8 months). The most common manifestations were mucocutaneous (86.4%), hematologic (63.6%), musculoskeletal (61.4%), and renal disorder (47.7%). There was at least one positive serologic test in 88.7%. Five patients (11.4%) had comorbidity, usually hypertension (9.1%). The initial lupus treatment consisted of moderate to high doses of glucocorticoids and hydroxychloroquine. Patients with life-threatening or organ-threatening disease, usually nephritis, received cyclophosphamide, azathioprine, or mycophenolate mofetil. One patient received rituximab. Fewer patients with nephritis received cyclophosphamide infusions during the first six months compared to the later six months. Most of the hospitalizations (34/36) occurred during the first six months and 22 of these were for diagnosis. Seven patients had more than one hospitalization and five (20%) had 30-day readmissions. mLLDAS was achieved by 15 (34.1%) and 30 (68.2%) patients at 6- and 12- months, respectively. Only one patient was in remission a year after diagnosis. Seven patients (15.9%) were assessed with organ damage, six (13.64%) of them at 6-months post-diagnosis. Organ damage was most commonly renal. Four (9.1%) patients died, all during their initial hospitalization. Conclusion In our population observed over a period of one year (2018-2019), there was a very low rate of remission (1/44, 2.3%), mLLDAS in 68.2%, and organ damage in 15.9%. Most of the hospitalizations (65%) were for the diagnosis of lupus and all deaths (9.1%) occurred during this first hospital confinement. We must intensify our efforts to (1) achieve earlier diagnosis, (2) deliver optimal lupus treatment and supportive care during the first lupus hospitalization, and (3) initiate early and persistent immunosuppressive treatment for nephritis to improve outcomes for our patents with SLE.
Lupus Erythematosus, Systemic ; Hospitalization ; Philippines

Background: Dermatomyositis - a rare autoimmune myositis – is a disease affecting primarily the skin and muscles which has been correlated with an elevated risk of solid tumors - commonly affecting the ovaries, breast, colon and nasopharynx. However, there is a rare association between dermatomyositis and pulmonary large cell neuroendocrine carcinoma such that in a thorough literature review of published material, only two cases have been reported internationally and none locally. Large cell neuroendocrine carcinoma - in itself, is also a rare malignancy representing only 1-3% of all primary lung carcinomas. Case Presentation: This is a case of a 53-year-old Filipino female, hypertensive, diabetic, dyslipidemic, hypothyroid - nonsmoker – who presented with an eight-month history of facial erythema, swelling of bilateral metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints, and erythema over extensor surfaces of the MCP and PIP joints. She had markedly elevated creatine kinase MM and positive anti-nuclear antibody for which she was prescribed prednisone, which she did not comply with. She lost weight and experienced severe abdominal pain. Abdominal imaging subsequently revealed multiple confluent abdominal and thoracic lymphadenopathy with histopathology of large cell neuroendocrine carcinoma (LCNEC). Peculiar to this case however is that despite being a lung carcinoma, the scan showed no pulmonary masses or nodules. Immunohistochemical stains of the lymph node were positive for neuroendocrine markers: pancytokeratin, synaptophysin, TTF-1 and negative for any mutation in the epidermal growth factor receptor. Her Ki-67, which is used as a prognostic factor and correlates with mitotic count - was 70% and PD-L1 tumor proportion score – a predictor of therapeutic effect - is 5-10%. She was subsequently diagnosed with dermatomyositis and pulmonary LCNEC. She has presently completed her 8th cycle of cisplatin and etoposide and has gained weight. Presently, her musculocutaneous lesions have resolved. However, a repeat PET scan was done still showing multiple confluent paraaortic, aortocaval, pericaval lymph nodes with no significant interval change from the first PET scan. Next generation sequencing had been requested showing DIS3 to be the gene altercation – however, as of this writing, no available therapeutic modalities are available to target this. Patient was nonetheless given Pembrolizumab for 3 cycles and subsequently expired due to complications of pneumonia. Conclusion Among published data, we herein present the third reported case of dermatomyositis associated with pulmonary large cell neuroendocrine carcinoma worldwide and the first reported case in the Philippines thereby contributing to the present medical literature. This case demonstrates two rare diseases associated with each other and exemplifies the need for an awareness of such disease entities. It demonstrates a rare case of LCNEC peculiarly without any pulmonary masses or nodules. It also illustrates the necessity in evaluating patients with dermatomyositis for their respective risk in terms of malignancy and other immunocompromised states. Lastly, it contributes to the knowledge on therapeutic options that may be given to patients presenting with both disease entities.
Dermatomyositis ; Lung Neoplasms

Objective: The coronavirus disease 2019 (COVID-19) pandemic has limited healthcare delivery for patients with chronic diseases, including Systemic Lupus Erythematosus (SLE). This study aims to describe the outcomes of patients with SLE in a national COVID-19 referral center in the Philippines. Methods: A review of records of all adult patients with SLE seen in the University of the Philippines-Philippine General Hospital (UP-PGH) from March 2020 to December 2021 was done. Data about patient characteristics, health encounters, and outcomes before and after the first visit during the study period were extracted. Descriptive statistics were employed. Results: Our population of 403 patients was predominantly young (mean age 34.53 ± 11.14 years), female, and unemployed. This consisted of 370 known cases of SLE, 92 were diagnosed in institutions outside UP-PGH, and 33 new patients. Over the 22-month study period, there were 2,093 medical encounters, most of which were teleconsultations (81.70%). During an average gap of 53.6 ± 26.7 weeks between the last consultation and the first visit within the pandemic study period, 84 patients (22.70%) discontinued at least one of their SLE control medications, 68 (18.38%) patients developed a lupus flare, and 79 (21.35%) were hospitalized for various reasons. On their return to the rheumatology clinic during the pandemic, 37.47% were in lupus flare, 28.29% needed to be hospitalized, and 20 died. However, 86.75% of flares were controlled. During subsequent health encounters, 48 patients had a new flare (43 of these were controlled) and 20 died. The most common reason for hospitalization (n=160) was lupus disease flare and the most common cause of death (n=40) was pneumonia. Sixty patients acquired COVID-19 infection from which most recovered and four died. Conclusion Audio teleconsultation was the most common method used by our lupus cohort to interact with their doctors during the pandemic. There was an average of a year-long interruption in medical care for 62.70%. More than a third developed a disease flare and 15% acquired COVID-19 but outcomes were good in more than 85%. Despite the challenges posed by the pandemic, the majority of our lupus cohort who were able to continue their treatment had favorable outcomes.
COVID-19 ; Philippines

Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Background: Seizures in patients with systemic lupus erythematosus (SLE) are uncommon but life-threatening; mortality rate is 25-29%. Seizure in a person with lupus may be due to lupus itself or other conditions. There are no published studies describing the causes and outcomes of seizures in Filipino patients with lupus. Objective: To describe the causes and outcomes of seizure in a cohort of patients with lupus seen at Philippine General Hospital. Methodology: We reviewed the medical records of patients with SLE) with a documented seizure and admitted between January 2016 and April 2019. History, physical examination and laboratory findings, and clinical course were obtained. Results: We included 29 patients with 31 seizure events. They were all women, mostly single, of low socio-economic status, and had poor functional capacity. Lupus was active in 77.4% (24/31), commonly with mucocutaneous or hematologic manifestations. Seizures were generalized in 87 % (27/31). Prior to seizure, one-third had headache, fever, and vomiting. There were no neurologic localizing signs. Twenty-four seizure events (77%) occurred among patients with active lupus; 16 (67%) was attributed to neuropsychiatric systemic lupus erythematosus (NPSLE) and eight (33%) to other causes: infection (tuberculous meningitis and septic encephalopathy), posterior reversible encephalopathy syndrome (PRES), uremia, arrhythmia, and eclampsia. Seven seizures in inactive lupus were not SLE-related. Mortality rate was 28%; infection was the most common cause. Seizure resolved in 97%. Mean duration of hospitalization was 26.7 days. Patients were discharged improved from 19 seizure events (18 patients); 14 had follow-up consultations, three were readmitted. There was no seizure recurrence within 30 days of discharge. There was improvement in functional capacity. Conclusion The most common cause of seizure was NPSLE, followed by infection. Despite high rates of complete seizure resolution, poor outcomes were noted in almost half of the patients. Prolonged hospitalization was common. A high rate of mortality was observed. Infection was the most common cause of mortality.
Lupus Vasculitis, Central Nervous System

Background: Rheumatoid arthritis (RA) is a chronic and progressive disease resulting in disability and poor quality of life. Patients’ knowledge (PxK) of disease can contribute to better disease control, reduced disability, and improved quality of life. Objective: The objective of this study was to determine the disease knowledge and functional disability of a cohort of patients with RA. Methods: We conducted a cross-sectional study among patients with RA at the University of the Philippines - Philippine General Hospital Arthritis Clinic. The subjects were recruited using convenience sampling over three months in 2019. We obtained demographic and disease characteristics, clinic attendance, patient knowledge, and functional status through chart review, a questionnaire, and a disability index. Descriptive statistics, 2-sample T-test, Pearson’s correlation, analysis of variance (ANOVA), and multiple linear regression analysis were used for data analysis. Informed consent was obtained before participation in the study. Results: Eighty percent (57/71) of recruited patients participated in the study. All were female, and the mean age was 51.6 years (±12.9). Most participants completed secondary education, were employed, had an average duration of RA of 8 (±6.8) years, had been consulting at the Arthritis Clinic for an average of 4.8 years (±3.8), and had moderate disease activity (49.1%). Most patients had American College of Rheumatology (ACR) class I or II functional status (50/57, 88%). The usual source of the patients' knowledge about RA was their physician. The mean knowledge score was 5.10 (±0.93) out of a perfect score of 9. Most patients were aware of their disease diagnosis and the non-communicable nature of RA. There was low awareness of the need for self-monitoring for disease flares or treatment adverse events and the extra-articular involvement in RA. The mean functional disability score was 6.65 ± 5.33, and the mean functional disability index (FDI) was 0.83 (±0.66). These correspond to mild functional disability (FD). There was no significant relationship between the total knowledge score and age, duration of disease, number of consultations in the past year, level of education, employment status, perceived level of disease knowledge, or the practice of asking their physician about illness. Perception of overall health status was associated with functional disability (p=0.001). Conclusion Most of the patients in our cohort of RA patients had a mild functional disability and low knowledge scores. The study identified the gaps in our patients’ knowledge of disease and its management. A re-evaluation of existing educational and treatment strategies will be beneficial to enhance disease knowledge and improve health outcomes.
Functional Status ; Arthritis, Rheumatoid

Objectives: Recent studies show that patients with young-onset gout present with visible tophi or nephrolithiasis on diagnosis. In the Philippines, where gout is prevalent, there is no published work on this subset of patients. This study presents the clinical characteristics of a cohort of Filipino patients with gout whose symptoms started at 30 years of age or younger. Methods: The case records of all patients who fulfilled the 1977 American College of Rheumatology (ACR) criteria for gout seen in four adult rheumatology services were reviewed. We selected those whose age of onset of gout was at 30 years or younger. The demographic characteristics, medical history, laboratory parameters, and presenting manifestations were described. Results: Six hundred sixty-nine records of patients with gout were reviewed; 101 (15%) fulfilled the young-onset gout criteria. The mean age of onset was 25±4.40 years (range 14-30), and the mean disease duration before diagnosis was 12.64±11.91 years. All of the patients were male and most were married; 76% were alcoholic beverage drinkers and 38% were smokers. A family history of gout was noted in 47%. Most patients (66%) were already on nonsteroidal anti-inflammatory drugs (NSAIDs), 24% on colchicine, and 14% on urate-lowering therapy before consult at the rheumatology clinic. By history, at onset, the most common pattern of joint involvement was monoarthritis (95%), affecting the ankles (60%), knees (52%), and 1st metatarsophalangeal (MTP) joint (51%). However, on the first rheumatology clinic visit, 34% of arthritis was polyarticular, more than 68% had more than three arthritis attacks per year, and there were tophi in 35%. The mean duration before visible tophi formation was 2.81±6.75 years. Around 21% had nephrolithiasis or a history thereof. The mean serum uric acid (SUA) was 9.18 mg/dL and the mean serum creatinine was 1.5 mg/dL. Thirty-seven percent had estimated glomerular filtration rate (GFR) <60 mL/min. Conclusion Young-onset gout was present in 15% of our patients and gout was familial in 47%. There was a delay in diagnosis of as long as ten years in most of the patients. On presentation at the rheumatology clinic, more than 34% had polyarticular arthritis, 35% had tophi, and 37% had low estimated GFR. This emphasizes the importance of awareness and prompt diagnosis to ensure correct treatment and prevention of complications
Gout

Objective: This study aimed to present four cases of Behçet's disease (BD) wherein recurrent calf pain was the first and prominent symptom experienced by the patients. Methodology: The case series described the clinical features, disease development, management, and outcomes of these patients. Written consent was obtained from each participant for the review and publication of their case. Results: All cases presented with chronic and recurrent calf pain, three of whom were bilateral. All four had skin changes on the lower extremities that may have contributed to leg pain. Only probing was a history of oral ulcers reported in all four; none had genital ulcers, eye abnormality, or sensory or motor deficits. All presented with elevated acute phase reactants. Based on the 2013 International Criteria for Behçet’s Disease (ICBD), a diagnosis of possible BD was made due to the presence of oral ulcers, skin lesions, and absence of other findings pointing to another connective tissue disease or systemic vasculitides. All four cases were given oral corticosteroids and colchicine, but two required the addition of methotrexate and azathioprine for control of symptoms. Conclusion A complaint of recurrent calf pain warrants inquiry and examination of the skin lesions over the affected area and other areas like the oral cavity and genital area. It also warrants consideration of a vasculitic process, especially in the background of oral ulcers and skin changes. In such cases, BD is the right differential diagnosis to be considered.
Pain