We describe a case of a 48-year-old woman who presented with acute respiratory failure due to diffuse alveolar haemorrhage and acute renal failure due to pauci-immune glomerulonephritis consistent with a new diagnosis of microscopic polyangiitis (MPA). The patient had a recent SARS-CoV-2 infection 6 weeks before MPA diagnosis and had stopped immunosuppression for her rheumatoid arthritis (RA) at that time. The patient was treated with pulse intravenous steroids, plasma exchange therapy and rituximab, which induced remission of her illness. This case highlights a timely dilemma of holding immunosuppression in a RA patient with low disease activity on combination therapy with SARS-CoV-2 infection, and the potential risk of developing an additional autoimmune disease, such as vasculitis, given their existing autoimmunity due to RA.
Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven–Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.
Background:Patients with rheumatic disease are at risk of developing glucocorticoid induced osteoporosis (GIOP) as many are prescribed systemic oral glucocorticoids as an adjunct to their maintenance therapy. Based on the 2017 ACR Guideline for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis, a good practice recommendation exists that “initial clinical fracture risk assessment should be performed as soon as possible, but at least within six months of the initiation of long term glucocorticoid treatment.1” Long-term glucocorticoid use is defined by duration of 3 months or greater. Fracture risk assessment should include dual energy-ray absorptiometry (DEXA) scan. Patients on greater than or equal to 2.5 mg of prednisone should be treated with an optimal dose of calcium and vitamin D and may benefit from oral bisphosphonate as primary prevention against GIOP if their fracture risk is moderate to high.1Objectives:The aim of this Quality Improvement Project is to assess the current status of provider implementation of GIOP recommendations in the rheumatology clinic. Ultimate goal is to improve osteoporosis prevention in the rheumatology clinic.Methods:We conducted a retrospective chart review of 60 patients in two outpatient rheumatology clinics. Clinic 1 follows patients with lower socioeconomic status and Clinic 2 follows patients with higher socioeconomics. Inclusion criteria were patients on long-term glucocorticoid use, defined as at least 3 months of corticosteroid use of at least 2.5 mg prednisone daily, as well as age less than 65. Females aged 65 or older were omitted to prevent overlap of the United States Preventative Taskforce recommendation for all women ≥ 65 years to be screened for osteoporosis with DEXA scans.2 DEXA scan orders, calcium and vitamin D prescriptions, and osteoporosis medication prescriptions were abstracted. After baseline data obtained, intervention of education of the rheumatology fellows and faculty, and internal medicine residents in the guidelines for GIOP prevention was implemented. In addition, a smartphrase in the electronic medical record was created for provider use when treating patients on chronic corticosteroids. Subsequently, two audit cycles were completed for retrospective chart review.Results:Upon completion of second audit cycle, there was no change in percentage of DEXA scan orders at Clinic 1, however there was a 10% overall improvement in DEXA scan orders in the Clinic 2.In terms of Calcium and Vitamin D prescriptions, there was an overall improvement in both clinics of 19.7% and 13.3% in Clinics 1 and 2 respectfully after the second audit cycle.Additionally, there was a 3.4% increase in osteoporosis medication prescriptions overall subsequent to the second audit cycle in Clinic 1. However in Clinic 2 there was an overall decrease in osteoporosis medication prescriptions of 6.6%.Clinic 1Prior to AuditAudit cycle 1Audit cycle 2Patient percentage without DEXA scan orders30%33.30%30%Patient percentage without Vitamin D/Calcium orders26.40%8.30%6.70%Patient percentage with osteoporosis medication orders23.30%8.30%26.70%Clinic 2Patient percentage without DEXA scan orders50%37.00%40%Patient percentage without Vitamin D/Calcium orders30%26.00%16.70%Patient percentage with osteoporosis medication orders23.30%11.10%16.70%Conclusion:Overall, the results of the intervention were strongest for improvements in Vitamin D and Calcium orders in both clinics. Improvements in DEXA scan orders and osteoporosis medications were present in Clinic 2 and not present in Clinic 1. This reveals continued efforts and education of providers need to be made for improvement in bone health monitoring.References:[1]Buckley, Lenore, et al. “2017 American College of Rheumatology Guideline for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis.” Arthritis & Rheumatology, vol. 69, no. 8, June 2017, pp. 1521–1537., doi:10.1002/art.40137.[2]Final Recommendation Statement: Osteoporosis to Prevent Fractures: Screening. U.S. Preventive Services Task Force. July 2019.Disclosure of Interests:None declared.
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