RESUMO
Myositis is a group of rare autoimmune disorders characterized by chronic inflammation of skeletal muscles that leads to a hallmark triad of muscle weakness, fatigue, and myalgia. Extra-muscular manifestations are sometimes seen and involve various organ systems, including the gastrointestinal (GI) tract. In this case series, two patients with polymyositis (PM) and dermatomyositis (DM), both of whom developed dysphagia as a complication of myositis, are discussed. Case 1 was a female with a known history of biopsy-proven dermatomyositis who presented with progressive peripheral edema and weakness affecting all extremities. Concurrently, she displayed symptoms of pneumonia and dysphagia associated with frequent spontaneous or self-induced vomiting to alleviate retrosternal discomfort. Esophagogastroduodenoscopy (EGD) revealed esophageal dilatation and an absence of a contractile response, consistent with myositis. Treatment comprised intravenous immunoglobulin (IVIG), mycophenolate, and lifestyle modifications, including dietary adjustments and maintaining an upright position postprandial. The second case was a female with muscle weakness and dysphagia. Video-fluoroscopic swallow assessment was significant for pharyngeal dysfunction without a sensory response to penetrated material, and the patient was at high risk of aspiration with any oral intake. The presence of pharyngeal dysfunction and dysphagia prompted treatment with IVIG, mycophenolate, and percutaneous endoscopic gastrostomy (PEG) tube placement. These cases have highlighted the upper GI complications observed in patients with myositis, accentuating the necessity for a personalized treatment approach. Timely intervention has shown promising results in symptomatic relief and improving patient outcomes. This emphasizes the importance of a multidisciplinary approach when addressing myositis-related upper GI manifestations.
RESUMO
Hypertrophic pachymeningitis (HPM) is a rare but extremely debilitating disease. It is even rarer for HPM to be seen in association with antineutrophil cytoplasmic antibody (ANCA)-negative vasculitis. In this case, we are presenting HPM that was diagnosed in a 28-year-old female patient who presented with worsening back pain. Imaging revealed dural-based enhancing masses affecting the thoracic spinal cord with compression. Infectious etiologies were ruled out and a total of three biopsies failed to show any evidence of granulomatous inflammation, malignancy, or evidence of immunoglobulin G4-related disease. ANCA was negative on repeated testing. The patient was managed with repeated short courses of steroids that resulted in symptomatic control as well as radiological stability of the disease. This is an extremely rare case of atypical presentation of spinal HPM that is likely associated with granulomatous and polyangiitis without other manifestations of the disease except for nasal septal perforation. This case is a supplement to a limited body of knowledge and established cases of HPM in ANCA-negative, ANCA-associated vasculitis.
