Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report

Abstract

Background: Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. Clinical case: A 56-year old African Black woman initially presented with systemic sclerosis (SSC) -myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia. Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal and she remained well 20 months after hospital discharge. Conclusion: Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge.

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].…”

Systemic sclerosis in sub-Saharan Africa: a systematic review

“…Spirometry results were reported in 184 of all SSc patients [ 6 - 8 , 10 , 13 , 14 , 17 , 19 , 24 , 26 , 27 , 29 , 38 , 39 , 41 , 45 , 47 - 49 , 85 ], either as percentage of predicted, or as a dichotomized result (normal/abnormal). 183 of these patients featured a reduced FVC.…”

“…There was no difference in HIV prevalence among patients with or without ILD [ 49 ]. Tuberculosis was diagnosed in 22 patients [ 13 , 29 , 38 ], of which 17 [77%] had latent tuberculosis [ 10 , 48 , 92 ] and the remaining 5 patients [23%] had active tuberculosis. Information about TB and HIV comorbidity was however not analysed in the vast majority of patients [98% and 92% respectively].…”

Rituximab Therapy in Polymyositis-associated Severe Dysphagia: A Case Report

“…5,6 The use of Rituximab in cases of refractory inflammatory myopathies was documented in several trials and a case report [overlapped systemic sclerosis (SSC)myositis]. 7,8 In this case, rituximab and IVIG was used for severe dysphagia requiring PEG feeding in a case of polymyositis.…”