Polymyositis and the Spectrum of Scleroderma Disorders

Abstract

Polymyositis (PM) is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK) elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl) and anti-Sjögren’s-syndrome A (SSA) antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation. LEARNING POINTS Polymyositis (PM) may be associated with connective tissue diseases such as systemic sclerosis, including its variant without skin involvement. Necrotizing muscle fibers are typically found in patients with overlap syndrome, in opposition to patients only with polymyositis. PM-Scl antibodies are associated to a good response to corticoids.

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

“…IIM can result in impairment of the oral [ 29 , 58 , 59 , 60 , 61 , 62 , 63 , 64 ], pharyngeal [ 7 , 23 , 24 , 27 , 29 , 31 , 32 , 33 , 44 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 , 88 , 89 , 90 , 91 , 92 , 93 , 94 , 95 ,…”

“…Reduced pharyngeal contractility can result in insufficient pharyngeal bolus clearance [ 139 ]. Consequently, myositis patients often show pharyngeal residue after swallowing [ 7 , 27 , 29 , 31 , 33 , 44 , 58 , 59 , 60 , 61 , 65 , 66 , 67 , 69 , 71 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 83 , 84 , 86 , 87 , 91 , 93 , 96 , 100 , 101 , 103 , 104 , 106 , 111 , 112 , 115 ]. These can impact on swallowing safety and ultimately cause aspiration, which is a frequently reported finding [ 33 , 78 , 81 , 86 ].…”

“…Various authors reported reduced or absent esophageal motility sometimes extending to the lower esophageal sphincter [ 29 , 38 , 114 ]. Most studies used manometry [ 24 , 29 , 38 , 59 , 85 , 90 , 98 , 105 , 114 , 117 , 118 , 119 , 122 , 129 , 130 ] some also VFSS, barium swallow or scintigraphy [ 62 , 73 , 81 , 89 , 99 , 104 , 120 , 121 , 124 , 125 , 127 ] to detect esophageal impairment.…”

“…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 ,…”

“…IIM can result in impairment of the oral [29,[58][59][60][61][62][63][64], pharyngeal [7,23,24,27,29,[31][32][33]44, and esophageal [24,29,38,59,62,73,81,85,89,90,93,95,98,99,101,[103][104][105]112,[114][115][116][117][118][119][120][121][122][123][124][125][126][127][128][129]…”