Resistant dermatomyositis in a rural indigenous Maya woman

Abstract

A 28-year-old indigenous Guatemalan woman presented with 7 months of progressive weakness and numerous dermatological findings. She initially sought care within the free government-run health system and was treated with oral steroids for presumed dermatomyositis. Her symptoms progressed, including severe dysphagia, hypophonia and weakness preventing sitting. She was lost to follow-up in the public system due to financial and cultural barriers. A non-governmental organisation tailored to the needs of Maya patients provided home intravenous pulse dose methylprednisolone in the absence of first-line biologicals. With longitudinal home-based care, she achieved symptom free recovery. The rising burden of chronic non-communicable diseases highlights shortcomings in health systems evident in this case, including lack of provider capacity, limited infrastructure to test for and treat rare diseases and poor continuity of care. We provide potential solutions to help care delivery in low-resource settings adapt to the demans of chronic disease control with particular attention to social determinants of health.

The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis

“…There are several articles reporting positive therapeutic effects of immunomodulatory medication on symptoms and/or on findings of objective swallowing evaluations. These include intravenous methylprednisolone pulse therapy [ 19 , 33 , 70 , 81 , 87 , 134 , 154 , 155 , 156 , 157 , 158 , 159 ], methotrexate [ 30 , 76 , 89 , 124 , 136 , 154 , 159 , 160 , 161 ], long-term prednisone/prednisolone [ 29 , 30 , 33 , 59 , 64 , 70 , 74 , 76 , 81 , 83 , 84 , 87 , 89 , 101 , 103 , 111 , 118 , 122 , 124 , 134 , 136 , 143 , 154 , 158 , 159 , 161 , 162 , 163 , 164 , 165 ,…”

“…There are several articles reporting positive therapeutic effects of immunomodulatory medication on symptoms and/or on findings of objective swallowing evaluations. These include intravenous methylprednisolone pulse therapy [ [173,177,178], hydroxychloroquine [30,118,154], tacrolimus [162,172], cyclophosphamide [83,170,172], mycophenolate mofetil [118,179], cyclosporine [123] and rituximab [155]. If dysphagia does not respond to medical therapy, it may be helpful to switch to another group of medication, e.g., from steroids to IVIG [24].…”