ABSTRACT
Background: Necrotizing myopathy has been previously described but was not included in the Peter and Bohan criteria until 2004, when immune-mediated necrotizing myopathy (IMNM) was distinguished from polymyositis (PM) based on immunologic and histopathologic differences. IMNM is currently a well-recognized autoimmune myopathy and represents up to 20% of these cases. Case: A 60-year- old female with biopsy-proven PM achieved sustained clinical remission with Rituximab. Her co-morbid conditions include hypertension, diabetes mellitus, and dyslipidemia. The patient noted a recurrence of gradual progressive, proximal muscle weakness and easy fatigability after receiving her first mRNA Covid-19 vaccine. Four months after onset of symptoms, CK Total was 9600 U/L. Rituximab was administered and muscle weakness and total CK levels (1247 U/L) improved within 10 days. She was prescribed rosuvastatin and fenofibrate for dyslipidemia within 7 days of completing the rituximab course. Two weeks later, proximal muscle weakness recurred. She became wheelchair-bound and experienced dysphonia. MMT score was 2/5 in proximal muscles and total CK total increased to 19,935 U/L. The patient received Methylprednisolone 500 mg IV once a day for 3 days. She had a good response with resolution of dysphonia and improvement of MMT to 4/5 on shoulder abduction and hip flexion on the 6th hospital day. She was discharged on oral methylprednisolone at 1 mg/kg/day. Muscle biopsy was consistent with an immune-mediated necrotizing myopathy, revealing necrotic fibers, intracellular macrophages, fatty infiltrates, irregular staining patterns on NADH stain with no evidence of endomysial inflammation, perifascicular atrophy, ragged red fibers, or rimmed vacuoles. Antibodiy against 3-hydoxy- 3- methylglutarylcoenzymeA reductase (HMGCR) result is pending but the other myositis-specific antibodies are negative.(including anti-SRP). Conclusion(s): IMNM is an autoimmune myopathy associated with anti-HMGCR and anti-SRP antibodies that clinically present similarly to polymyositis. The temporal occurrence of worsening muscle weakness with initiation of statin therapy make statin toxic myopathy or immune mediated necrotizing myopathy as diagnostic considerations. This case emphasizes the need to re-evaluate the etiology of new onset muscle weakness in patients with idiopathic inflammatory myopathy and highlights the role of myositis-specific antibodies and muscle biopsy in confirming the diagnosis.
ABSTRACT
Background: Telemedicine became the emergent means of providing and continuing medical care due to the COVID 19 pandemic. This study aims to evaluate the knowledge, perception, and satisfaction with the use of telemedicine among patients with rheumatic diseases. An understanding of our patients' experiences can be utilized to provide access to care, improve gaps in delivery of care, and improve healthcare disparities. Method(s): Filipino patients with rheumatic disease who had telehealth visits between June 2020 and August 2021 in St Luke's Medical Center Outpatient Department participated in an online survey. Information on demographics, diagnosis, knowledge and experience on telemedicine, and perspectives on benefits and limitations of telemedicine were collected. Result(s): There were 70 respondents: 52.9% with SLE, 25.7% with RA, 10% with osteoarthritis, 5.7% with psoriatic arthritis, 2.9% with scleroderma. Results showed that 64.3% are familiar with the use of video conference platforms. Facebook messenger was the most used (85.7%). Half of respondents have used telemedicine on their own, while 33% required assistance. The remaining respondents have not used telemedicine due to lack of experience or awareness on how to proceed with consults. The reasons for using telemedicine were restrictions of the pandemic (82.9%), limited access to clinics (31.4%), and disability (1.4%). Most remain satisfied with telemedicine (75.7%), 50% of patients stated that telemedicine was comparable to an in-clinic visit, and 85.7% (N = 60) would recommend its use. Conclusion(s): Filipinos with rheumatic disease are knowledgeable on online platforms and telemedicine, however, it is important to note the digital divide. Patients need assistance and improved awareness on accessing remote care. Providing continuity of healthcare can lead to less complications and better outcomes despite pandemic restrictions. There is an overall favorable satisfaction for care. Half the respondents remain satisfied with telemedicine. Rheumatologists need further studies on benefits and outcomes on providing remote healthcare.
ABSTRACT
Introduction: About 15% of patients with interstitial lung disease (ILD) have an underlying systemic rheumatic disease and among them, approximately 2-5% have Systemic Lupus Erythematosus (SLE). Etiology is still unknown but current hypotheses of pathogenesis include inflammatory response to environment pathogens causing influx of inflammatory cells to interstitial and alveolar spaces leading to alveolar epithelial damage. ILD can occur anytime during the course of SLE but on average it develops in patients with disease duration of 10 years and symptoms are non-specific. Usual treatments include systemic steroids, mycophenolate mofetil and cyclophosphamide. Case: This is a case of a 54 year-old female, Filipino with one-month history of productive cough accompanied by episodes of undocumented fever and dyspnea on exertion. Work-ups for COVID-19 including RT-PCR test were done and yielded negative results. Further work-ups were done including highresolution computed tomography (HRCT) of the chest which showed reticular opacities, honeycombing and traction bronchiectasis in both lungs predominantly in lower lobes, pleural thickening and pericardial effusion. Parenchymal findings were consistent with interstitial lung disease. Pulmonary function test showed moderate restrictive ventilator defect with reduced DLCO. ANA and anti-dsDNA results were 1:640 speckled pattern and 41.2 respectively and a score of 13 using EULAR Criteria for SLE. She was given Methylprednisolone 65 mg IV once daily, which she did not tolerate. There was worsening of dyspnea and desaturation, hence she was intubated. Treatment for SLE-ILD was shifted to Intravenous Immunoglobulin (IVIg) 25 g and she was able to receive total of five doses. She was extubated after 11 days and was discharged on the 37th hospital day. Discussion: Diagnosis of SLE-ILD is generally clinical, based on presence of extrapulmonary and serologic evidence of SLE combined with HRCT findings confirming ILD and exclusion of other potential causes. Aside from the usual treatment using systemic steroids, severe cases can be managed using Rituximab while refractory cases are managed with IVIg and plasmapheresis. To date, there are no available practice guidelines for the management of SLE-ILD. The rest of the therapeutic approaches have been extrapolated from those utilized in systemic sclerosis due to the similarity of interstitial involvement. Literatures documenting success of IVIg in management of SLE-ILD are also limited;hence this case was presented to document successful response to IVIg treatment in a patient diagnosed with SLE-ILD.