Anti-Myeloperoxidase (MPO) associated Vasculitis in a young Filipino Male with Bronchiectasis: A case report

Background@#ANCA-associated vasculitis and its subtypes have been associated with pulmonary manifestations, with bronchiectasis being a unique clinical presentation.@*Case Summary@# We report the case of a 26-year-old Filipino male who presented with progressive dyspnea, neuropathic pain, and purpuric rash. Diagnostic evaluation revealed upper lobe bronchiectasis and lower lobe pneumonia, as well as hematuria and proteinuria. ANCA-associated vasculitis (AAV) and tuberculosis were considered. There was improvement of dyspnea, cough and rashes with antibiotics, glucocorticoids (GC), and anti-TB coverage. However, neuropathic pain progressed to the upper and lower extremities with development of weakness. Anti-myeloperoxidase (MPO) Anti-Neutrophil Cytoplasmic Antibody (ANCA) was positive, Electromyography-Nerve Conduction Velocity (EMG-NCV) revealed diffuse sensorimotor axonal polyradiculopathy of both upper and lower extremities. Cyclophosphamide was then given. The patient gradually regained his motor strength while sensory deficits persisted. He was referred to rehabilitation medicine for physical therapy and eventually discharged. This case highlights vasculitis as an associated extrapulmonary manifestation of bronchiectasis, and the possible role of bronchiectasis in the immune-mediated pathogenesis of ANCA- associated vasculitides.

Clinical factors associated with inadequate treatment response to methotrexate among patients with rheumatoid arthritis in a tertiary referral hospital

Objective@#The study aimed to determine patient-related factors associated with inadequate treatment response of patients with rheumatoid arthritis (RA) to methotrexate (MTX). @*Methodology@#We reviewed the medical records of patients with RA seen at the rheumatology outpatient clinic of Philippine General Hospital; dichotomized into those with adequate and inadequate treatment response to MTX. Clinical data including age, gender, comorbidities, smoking status, tender joint counts, disease activity score-28 (DAS28), and rheumatoid factor (RF) positivity were compared between the two groups. We used univariate binary logistic regression to determine whether these clinical factors were significantly associated with inadequate treatment response. @*Results@#Majority (47/77 or 61%) of patients have inadequate treatment response to MTX. There were no significant differences between the two study groups in terms of age, tender joint count, DAS28, sex, smoking status and RF positivity. These factors also failed to show significant association with inadequate treatment response. @*Conclusion@#Age, sex, smoking status, duration of disease, initial DAS28, RF positivity and tender joint counts are not significantly associated with inadequate treatment response. A larger sample size is needed to estimate the prevalence of inadequate treatment response to MTX and derive a predictive model for treatment response.

Functional disability of Filipino patients with hand Osteoarthritis seen at a Rheumatology outpatient clinic of a Tertiary Government Hospital

Introduction@#Osteoarthritis (OA) is a common joint disease which may cause functional limitation in daily activities. The aim of this study is to determine disability of patients with hand osteoarthritis (HOA) using the Disability of Arm, Shoulder and Hand (DASH) questionnaire and to determine association of risk factors to the development of HOA. @*Methods@#This is a cross-sectional study done in a tertiary government hospital. Patients seen at the rheumatology outpatient clinic aged 40 years and above, diagnosed with primary OA of hands, knees, hips and spine based on American College of Rheumatology criteria and who gave written informed consent, were included. Patient characteristics were extracted. Descriptive statistics were used. Those with HOA were compared to those without HOA, as to biomechanical stress, menopause, body mass index (BMI) and family history. They were made to complete the DASH questionnaire. Data was analyzed using chi-square and logistics regression analysis to determine association of risk factors for HOA. @*Results@#One hundred fifty patients with primary OA were included; 61.3% were between 60-79 years, 92% were female. Forty-one percent were obese, 55.3% had family history of OA. There were 88 cases of HOA (59%). Mean age at onset is 58.2±8.8 years, mean age at diagnosis is 61.8±9.6 years. The most common complaints were stiffness and pain (60.3%), stiffness only (29.5%) and pain only (10.2%). The average DASH is 28.7±15.6 (mild disability), seen in 59.1%, moderate disability in 37.5% and severe in 3.4%. Among the risk factors for the development of HOA, there was a trend for menopause, BMI and biomechanical stress to increase risk of HOA. Among the biomechanical factors, cooking, laundry and cleaning showed a trend toward increasing risk two-fold.@*Conclusion@#This study showed that half of patients with primary OA in the study had HOA that was mildly disabling in a majority, and moderately disabling in 1/3 of cases. There may be correlation with biomechanical factors in its development. Other important risk factors for its development need to be further studied in a larger cohort of patients.

Cardiovascular risk factors in Filipinos with Rheumatoid Arthritis included in the Rheumatoid Arthritis Database and Registry (RADAR)

Introduction@#Rheumatoid arthritis (RA) is the most common autoimmune inflammatory arthritis of unknown etiology. Cardiovascular disease (CVD) remains a major problem for these patients. This paper aims to describe the presence of cardiovascular risk factors among filipino patients with rheumatoid arthritis seen in the Philippine General Hospital Rheumatology outpatient department. This will also serve as a baseline database for patients with cardiovascular risk factors for future studies on the impact of rheumatoid arthritis on cardiovascular morbidity and mortality. Objective: to describe the presence of cardiovascular risk factors among filipino patients with rheumatoid arthritis seen in the Philippine General Hospital Rheumatology outpatient department included in the Rheumatoid Arthritis Database and Registry (RADAR)@*Methods@#Cases entered in the study were taken from the RADAR. Included patients were those worked up for traditional and non-traditional cardiovascular risk factors. Demographic data, traditional and nontraditional cardiovascular risk factors and management for RA and CVD were extracted. Descriptive statistics were applied. This study is approved by the Institutional Review Board.@*Results@#Ninety eight patients were included in this study. Ninety four percent were female with mean age at diagnosis of 49.95±10.17 (SD) years and mean disease duration of 63.01 months. For traditional cardiovascular risk factors: 18% were smokers; 34% (24/71) were obese; mean BMI was 23.85±4.60 (SD) kg/m2; 39% (38/98) had hypertension of which 87% were on antihypertensive medication; 19% has impaired fasting glucose (IFG) or diabetes and 55% had dyslipidemia but only 53% and 33% were on oral hypoglycemic agents and statins, respectively. For non-traditional CV or diseaserelated risk factors, 20% still had high disease activity and 65% had elevated sedimentation rate (mean 45.58±18.36 (SD) mm/hr) on latest consult. Eighty seven percent were taking methotrexate but only three percent were on biologic agents.@*Conclusion@#This study shows the presence of important traditional risk factors such as hypertension, diabetes, dyslipidemia, and obesity in this population. Significantly, more than half the cases have dyslipidemia. In addition, RA disease activity was high to moderate. The combination of traditional and disease related risk factors for cardiovascular disease is ominous and warrants aggressive treatment. In addition, patient education and weight control should be emphasized. It is recommended that this cohort be followed up prospectively.

Methotrexate toxicity and associated risk factors in Filipino patients with Rheumatoid Arthritis included in the Rheumatoid Arthritis Database and Registry

Introduction@#Rheumatoid arthritis (RA) is a chronic autoimmune disease that is severely debilitating with a prevalence in the Philippines of 0.17-0.4%. This study aims to determine rate of methotrexate (MTX) toxicity, identify risk factors and comorbid conditions predisposing to toxicity and describe management of MTX toxicity.@*Methods@#Rheumatoid arthritis (RA) cases from the Rheumatoid Arthritis Database and Registry (RADAR) diagnosed by the 1987 ACR criteria receiving MTX monotherapy or combination disease modifying anti-rheumatic drugs (DMARDs), with at least one dose of treatment, were included. Patients were grouped into those with and without adverse events (AE). Disease activity was measured using DAS 28-ESR. Baseline characteristics, duration of use, dose, concomitant drugs and all toxicities were listed. Management of AEs were described. Independent t-test and Mann-Whitney U test were used for numerical data and Chi-square and Fisher’s exact test for continuous data.@*Results@#One hundred ninety four patients are included, with 95% females, age 35-64 years, disease duration of 0.2-10 years. Eighty three percent are on methotrexate monotherapy. Fifty cases (25.77%) all with dose of 8.75±2.5 had AEs: hepatotoxicity (52%), gastrointestinal (24%), hematologic (14%), dermatologic (8%), pulmonary (6%). Risk factors directly correlated with toxicity were older age (p=0.024), disease duration (p=0.024 ), dose (p<0.000), duration of use (p≤0.001), anemia (p=0.038) and osteoarthritis (p=0.011).Management included dose reduction (52%), dose retention with close monitoring (26%), addition of (24%) or shift to (22%) other DMARDS. Folate dose was increased in all cases.@*Conclusion@#Methotrexate (MTX) toxicity rate of RA patients from the RADAR is similar to those in literature. While dose reduction is the main management strategy, some patients’ doses were maintained while others were shifted to other DMARDS.

Disease characteristics of Behcet’s Disease among Filipino patients seen in Rheumatology Clinics

Introduction@#Behcet's disease (BD) sometimes called behcet's syndrome or silk road disease is an immunemediated systemic vasculitis. This condition remains a clinical challenge for physicians. There are many reports, mostly case series and nationwide surveys, on clinical manifestations of BD from different parts of the world. In the Philippines where BD is rare and underreported, physicians might not be familiar with the clinical manifestations of this disease. The aim of this research is to describe the disease presentation of BD among Filipinos to increase awareness and avoid delay in diagnosis which might pose a threat for the development of irreversible, sometimes fatal complications.@*Methods@#A manual search was done for medical records with diagnosis of BD in the clinics of rheumatology staff of PGH. The diagnosis of BD was based on the 2006 International Criteria for BD. We noted the demographic data, clinical manifestations, results of ancillary procedures, treatment and outcomes. The study follows a descriptive design. @*Results@#There were 31 patients with the diagnosis of BD found from the manual search. Most of them were female (77%). The mean age at diagnosis was 38.6 years ± 10.4 (SD) and the mean time duration from onset of first symptom to diagnosis was 41 months (range three to 180 months). Three patients had a family member who also had BD (10%). The most common features of the disease were oral ulcers (94%), ocular manifestation (68%), and cutaneous disease (65%). The pathergy test was positive in 17%.The most common treatments prescribed were oral steroids (74%), colchicine (58%), and NSAIDs (48%). There was symptom control or improvement in a third of patients but there were still symptom recurrence in some. Thirteen patients (42%) had recurrent oral ulcerations while 23% had recurrence of skin lesions. Two of the patients (six percent) developed blindness. There was no death recorded. @*Conclusion@#There is an average delay of three years in the diagnosis of BD that hinders appropriate early treatment. Moreover, BD remains to be a clinical challenge for physicians. While a third of the cohort had good outcomes, half still had symptom recurrences and the occurrence of blindness in two patients underlines the potential of the disease to disable. We recommend expansion of the cohort to include the BD patients of other rheumatologists in the Philippines to have an idea on the actual prevalence and incidence of how this uncommon disease in our locality, and to have a better understanding of its clinical presentation and disease management in our country.