Table 1 Clinical features and investigations
From: Gastroparesis associated with Juvenile Dermatomyositis
| Â | Case 1 | Case 2 |
|---|---|---|
Diagnosis | Juvenile dermatomyositis with overlap features | Juvenile dermatomyositis |
Age at onset of gastric dysmotility symptoms | Seventeen | Thirteen |
Age at diagnosis of connective tissue disorder | Eleven | Sixteen |
Features of dermatomyositis | Rash, proximal weakness, raised muscle enzymes (CK 6926 units/l, ALT 126 units/l) | Rash, muscle pain, raised muscle enzymes (ALT 143 units/l LDH 1097 units); abnormal muscle MRI |
Other clinical features | Sialadenitis Pulmonary fibrosis | Polyarthritis |
Gastrointestinal features | Vomiting Abdominal pain Weight loss Required NJ feeding | Recurrent vomiting and abdominal pain |
Autoantibodies | ANA, 1/640 RF, Anti Ro, La, Sm, RNP positive | ANA 1/640. |
Gastrointestinal investigations | Gastric emptying study grossly delayed H. Pylori negative barium normal | Normal endoscopy Coeliac screen negative H. Pylori negative |
Treatment | Prednisolone Mycophenolate Mofetil, Intravenous immunoglobulin | Methotrexate |
Response to treatment | Normal gastric emptying study | Reduced frequency and severity of symptoms |