Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus.

OBJECTIVE: To determine the association of systemic lupus erythematosus disease activity index (SLEDAI) score in pediatric onset SLE (p-SLE) with clinical and laboratory parameters. METHODS: This cross sectional observational study was conducted at Division of Rheumatology, Fatima Memorial Hospital, Lahore from November 2018 to January 2019. Total 23 patients diagnosed with p-SLE having onset of symptoms at ≤ 18 years of age, irrespective of their current age at presentation, of either gender, fulfilling criteria of 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were enrolled. Patients' clinical symptoms and laboratory parameters were reviewed, SLEDAI scores were calculated. Collected Data were entered in proforma and analyzed on SPSS version 23. RESULTS: There were 91.3% females. Mean age at diagnosis was 11years ± 4years. At presentation patients had hematological involvement 69.6% followed by mucocutaneous symptoms 65.2% and renal involvement 21.6%. ANA by IFA was positive in all, while anti-ds-DNA was positive in 78.3% patients. SLEDAI score was ≥6 in 87% patients, average SLEDAI score was higher in patients with renal involvement (p=0.06). Elevated ESR (r=0.48, p=0.02), Anti-dsDNA (r=0.44, p=0.05) and low complement levels (p=0.03) were significantly positively correlated, while hemoglobin (r= -0.43, p=0.04) was negatively correlated with the SLEDAI score. CONCLUSION: In this study, patients with Lupus Nephritis had high SLEDAI scores. Elevated Anti-dsDNA titer, ESR, low complement levels and hemoglobin were significantly associated with high SLEDAI scores. We recommend that SLEDAI score should be calculated in p-SLE patients for stringent disease monitoring and treatment.

Pattern of initial clinical manifestations of systemic lupus erythematosus in a tertiary care hospital.

OBJECTIVE: To determine the pattern of initial clinical manifestations of Systemic Lupus Erythematosus (SLE) and to compare these features with those recorded elsewhere in Pakistan. METHODS: This cross-sectional, descriptive study was performed in the Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan, from November 2015 to January 2016. Sixty one patients of SLE diagnosed as per ACR (American College of Rheumatology) 1982 revised criteria, were enrolled. The patients were evaluated for the initial clinical manifestations of SLE. The information was collected on a specially designed proforma and analyzed by using SPSS version 17. RESULTS: Out of 61 patients, 49 (80.3%) were females and 12 (19.7%) males, showing a female to male ratio of 4:1. The mean age of patients was 26.2 ± 7.9 years. Fatigue was the most common presenting feature in 56 (91.8%) patients, followed by joint pains in 55 (90.2%) and fever in 54 (88.5%). Renal involvement was found in 46 (75.4%). Comparison of these presenting features was made with other studies carried out in Northern Pakistan (Islamabad) and in central Punjab (Pakistan). There were statistically significant differences in fever, fatigue and arthritis between our patients and the other two above mentioned study groups. However, comparison of renal manifestations showed significant difference only with Islamabad study, and not with previous study from central Punjab. CONCLUSION: In this study, majority of patients presented with combination of fatigue, fever, rash and arthritis. Almost three-fourth of patients had renal manifestations at initial presentation. Therefore, it is important for clinicians to have high index of suspicion for SLE, when patients present with above symptoms.

Adherence to Methotrexate therapy in Rheumatoid Arthritis.

OBJECTIVE: To determine adherence to methotrexate (MTX) therapy in patients with Rheumatoid Arthritis (RA) and to identify factors that promote either adherence or non adherence. METHODS: One hundred Rheumatoid Arthritis patients on MTX for at least two months were enrolled. Questionnaire was completed by direct interview. Details recorded were, demographics (age, sex, education, monthly income), disease duration, duration on MTX and current dose. Disease Activity Score on 28 joint counts (DAS 28) at the current visit, concomitant drugs taken and number of doses of MTX missed in the previous 8 weeks were noted. Non adherence was defined as omission of any three or more prescribed doses of MTX in previous 8 week. Patients were asked for the factors that motivated their adherence to MTX as well as factors for non adherence. Presence of side effects due to MTX was also recorded. RESULT: Non adherence was found among 23% of cases. Patients of low socioeconomic group (p <0.0001) and on MTX for longer duration (p <0.001) had higher non adherence. Non adherent patients had significantly higher disease activity as measured by DAS 28 (p<0.001). Good counseling and education by the doctor was a strong predictor of adherence (p <0.001). Lack of affordability (p <0.001); lack of availability at local pharmacy (p <0.001); lack of family support (p <0.001) and lack of awareness regarding need and importance of MTX (p < 0.001were found as significant factors for non adherence. CONCLUSION: MTX non adherence in RA is noted in about one fourth of study group. Various economical and social issues lead to non adherence but good patient education and counseling by doctor could promote adherence in this study group.

It's About Time: A Study of Rheumatology Patient Consultation Times.

OBJECTIVE: A study was conducted evaluating the process of a rheumatology consultation. METHODS: Data on consultation times was obtained from 100 patient processes over three months. Prior to data collection, unstructured interviews were conducted with the entire staff of the rheumatology clinic in Shalamar Hospital, to understand the consultation process. Based on this, consultation was divided into distinct segments (vitals, history and examination, specialist registrar consultation, specialist consultation, documentation and exercise/prescription handing over) and data was collected for the time taken for the patient to complete each segment. Designation of the personnel conducting the process, diagnosis, current visit number and general notes were also recorded. RESULTS: Patients with rheumatoid arthritis (RA) consulted for an average time of 33.4 and 27.4 minutes for new and established patients respectively in our observations. Patients with systemic lupus erythematosus (SLE) on the other hand spent 34.5 and 37 minutes for new and established patients respectively. The greatest time spent during any segment of the consultation was during documentation, which averaged 10 minutes per patient. CONCLUSION: Our study found that consultation times at Shalamar Hospital's rheumatology clinic align with international guidelines. Implementing a triaging method could optimize resource allocation, while entrusting specialist nurses with stable patient follow-ups could enhance patient flow and provision of health education.

An Atypical Initial Manifestation of Systemic Lupus Erythematosus: Lupus Enteritis Accompanied by Intestinal Pseudo-Obstruction and Bilateral Hydronephroureter.

Systemic lupus erythematosus (SLE) is a systemic, autoimmune, multisystem disease. Lupus enteritis accompanied by intestinal pseudo-obstruction (IPO) is a serious and rare initial manifestation that can lead to high mortality and morbidity in case of delay in diagnosis and treatment. Here, we present a very complicated case of a 36-year-old female Pakistani patient with lupus enteritis accompanied by IPO and bilateral hydronephroureter. The patient had a three-month history of fever, weight loss, recurrent diarrhea, vomiting, alopecia, and photosensitivity. She had a malar and discoid rash, with signs and symptoms of IPO and neuropsychiatric lupus. Her labs revealed positive anti-nucleosome antibodies (8 U/mL), anti-Ro antibodies (100 U/mL), and anti-La antibodies (53 U/mL); equivocal anti-dsDNA antibodies (7 U/mL) and anti-Sm antibodies (7 U/mL); direct Coomb's positive hemolytic anemia; raised C-reactive protein and erythrocyte sedimentation rate levels; low complement (C3 and C4) levels; and pyuria. IPO was evident on abdominal X-ray and CT scan. Her Systemic Lupus Erythematosus Disease Activity Index was 24, indicating severe disease flare. She was treated with intravenous methylprednisolone, hydroxychloroquine, and intravenous 500 mg cyclophosphamide. Her lab parameters and clinical mini-mental score improved, from 0/30 to 18/30. She was discharged on oral prednisolone 0.5 mg/kg/day, hydroxychloroquine, trimethoprim-sulfamethoxazole (prophylaxis for Pneumocystis jirovecii pneumonia), and mineral and vitamin supplements. She was followed up on the 15th day of discharge for the next dose of cyclophosphamide, and her clinical and lab parameters were normal at that time with gradual improvement in cognition. Lupus enteritis with coexisting IPO and bilateral hydronephroureter poses a diagnostic and therapeutic challenge because of atypical and uncommon manifestations of lupus and overlapping features with intestinal tuberculosis and other inflammatory bowel conditions.

Prevalence of inflammatory back pain and radiographic axial spondyloarthritis in a semi-urban community of Lahore, Pakistan.

AIMS: To determine the prevalence of inflammatory back pain (IBP) and radiographic axial spondyloarthritis (SpA) in a semi-urban community of Lahore, Pakistan. METHODS: This cross-sectional household survey was designed as per the Community Oriented Program for the Control of Rheumatic Diseases (COPCORD) model. In Phase 1, the subjects were interviewed for musculoskeletal (MSK) pain in the last 7 days by clinical assistants. In Phase 2, physiotherapists identified subjects with spinal/back pain and interviewed for Assessment in Spondyloarthritis International Working Group (ASAS) criteria for IBP. In Phase 3 subjects having IBP or chronic back pain (CBP) with an age at onset ≤45 years, were assessed and further investigated. RESULTS: A total of 4922 subjects with a mean age of 35.3 ± 14.5 years, including 2770 (56%) women were surveyed in Phase 1. MSK pain in last 7 days was reported by 1407 (28.6%) of whom 1034 (21%) had spinal pain. The ASAS criteria for IBP were met in 329 (6.7%, 95% CI 6.0-7.0). In Phase 3, 222 with IBP and 83 having CBP with age at onset ≤45 years were evaluated. Out of this total of 305, 144 (2.9%) were confirmed to have IBP by rheumatologists as per at least 1 of the 3 criteria. ASAS criteria were met in 107 (2.2%, 95% CI 1.8-2.6). ASAS criteria for radiographic axial SpA were met in 47 (1%, 95% CI 0.7-1.3) of the surveyed population. CONCLUSION: Inflammatory back pain was reported in 6.7% by physiotherapists, confirmed in 3% by rheumatologists. The prevalence of radiographic axial SpA was 1%.

A rheumatology curriculum in Pakistan for empowering family physicians and fighting disability.

INTRODUCTION: Pakistan has a population of over 200 million with only 75 trained rheumatologists. To address the needs of rheumatology care, it is of paramount importance to train the primary care physician as a first line of defense. METHODS: The project "Empowering Family Physicians; fighting disability" was the recipient of a 2018 ILAR grant. This project began with development of an evidence-based curriculum using ACR Rheum2learn modules along with guidelines from international societies. A blended learning approach was chosen with nine online learning modules sandwiched between two face-to-face sessions. Participants' assessment entailed quizzes, clinical scenarios, and portfolio development all completed online, while face-to-face sessions relied upon power-point presentations and an objective structured clinical examination. Course impact was assessed with pre-course and post-course questionnaires. Overall perception of the training was evaluated through candidate feedback. RESULTS: Participants were enrolled from across the country totaling 48 health care providers (44 family physicians and 4 allied health professionals). The adherence to face-to-face sessions was 82.5% and 63.6% for the online component. The mean score for post-course assessment (mean = 2.369, SD = 0.3425) was significantly higher than for the pre-course assessment (mean = 1.792, SD = 0.4838) with statistically significant difference of, t (12) = - 7.756, p < 0.0001 (confidence interval: - 0.7390 to - 0.4149). The perception of the strategy was positive with 80% strongly satisfied with the workshops and presentations. CONCLUSION: Empowering family physicians by training them in rheumatology care can be an effective tool to fight unmet needs in access to musculoskeletal health care. We plan to offer a shortened version of the course at regular intervals.Key Points• Pakistan has a huge shortage of rheumatology care with only 75 rheumatologists caring for a population of over 200 million.• To improve access to rheumatology care,the "Empowering Family Physicians; Fighting disability" course was launched in 2018 with the help of anILAR grant.• A blended learning approach comprising of 9 online modules sandwiched between two face-to-face sessions was chosen.• A statistically significant difference between pre- and post-courseself-assessment of participantssuggests that the courseis an effective tool for teaching Family Physicians.