A 71-year-old male initially presented with vocal cord palsy and underwent tracheostomy. After thorough examination, urogenital dysfunction, orthostatic hypotension, and Parkinsonism were found, which led to the diagnosis of multiple system atrophy (MSA). After the tracheostomy, bi-level positive airway pressure ventilation was required during the night due to nocturnal hypoxemia. Night-time hypoxemia is related to central sleep apnea, which is one of the manifestations of MSA. This is the first case of MSA manifested by bilateral vocal cord palsy as an initial sign in Korea. This case supports the notion that MSA should be taken into consideration when vocal cord paralysis is observed.
Aged ; Airway Obstruction ; Anoxia ; Diagnosis ; Humans ; Hypotension, Orthostatic ; Korea ; Male ; Multiple System Atrophy* ; Parkinsonian Disorders ; Sleep Apnea, Central ; Tracheostomy ; Ventilation ; Vocal Cord Paralysis*

Macrophage activation syndrome (MAS) is a secondary hemophagocytic lymphohistiocytosis caused by autoimmune diseases, such as systemic lupus erythematosus (SLE). It is characterized by fever, cytopenia, coagulopathy, hepatosplenomegaly, elevated liver enzyme, and high ferritin, typically combined with hemophagocytic histiocyte proliferation in the bone marrow. Here, we report a case of MAS in a patient with SLE treated successfully by tocilizumab. She was transferred to our hospital due to persistent fever of unknown origin. Initial blood tests revealed cytopenia, elevated liver enzyme, and high ferritin. Bone marrow histology revealed the presence of hemophagocytic histiocytes. The patient was initially treated with high dose corticosteroids; however, fever and cytopenia were not controlled. Additional treatments with cyclosporine, intravenous immunoglobulin, and rituximab were applied consecutively, but the fever and cytopenia persisted. Symptom resolution was finally achieved following treatment with tocilizumab, resulting in rapid improved of fever, and resolution of pancytopenia within 2 months.
Adrenal Cortex Hormones ; Autoimmune Diseases ; Bone Marrow ; Cyclosporine ; Ferritins ; Fever ; Fever of Unknown Origin ; Hematologic Tests ; Histiocytes ; Humans ; Immunoglobulins ; Liver ; Lupus Erythematosus, Systemic* ; Lymphohistiocytosis, Hemophagocytic ; Macrophage Activation Syndrome* ; Macrophage Activation* ; Macrophages* ; Pancytopenia ; Rituximab

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.
Glucocorticoids ; Humans ; Immunoglobulins ; Inflammation ; Methylprednisolone ; Middle Aged ; Prednisone ; Retroperitoneal Fibrosis*

Necrotizing pancreatitis is associated with high rates of morbidity and mortality. Managing necrotizing pancreatitis is challenging, and minimally invasive treatment modalities recently replaced traditional open necrosectomy. Percutaneous catheter drainage and endoscopic necrosectomy are now widely used because they are less invasive, safer, and can more effectively remove necrotic materials. Various methods and novel techniques have been introduced to manage walled-off necrosis. Herein, we report a case series of patients with necrotizing pancreatitis who were successfully treated using fully covered esophageal metal stent and endoscopic necrosectomy via the percutaneous approach. Percutaneous endoscopic necrosectomy using a fully covered esophageal stent is an effective endoscopic treatment for patients with walled-off necrosis that extends along both sides of the paracolic gutter, reduces the number of necrosectomy sessions, and improves disease status faster than conventional treatment.
Catheters ; Drainage ; Humans ; Mortality ; Necrosis ; Pancreatitis ; Stents*

A 71-year-old male initially presented with vocal cord palsy and underwent tracheostomy. After thorough examination, urogenital dysfunction, orthostatic hypotension, and Parkinsonism were found, which led to the diagnosis of multiple system atrophy (MSA). After the tracheostomy, bi-level positive airway pressure ventilation was required during the night due to nocturnal hypoxemia. Night-time hypoxemia is related to central sleep apnea, which is one of the manifestations of MSA. This is the first case of MSA manifested by bilateral vocal cord palsy as an initial sign in Korea. This case supports the notion that MSA should be taken into consideration when vocal cord paralysis is observed.
Aged ; Airway Obstruction ; Anoxia ; Diagnosis ; Humans ; Hypotension, Orthostatic ; Korea ; Male ; Multiple System Atrophy ; Parkinsonian Disorders ; Sleep Apnea, Central ; Tracheostomy ; Ventilation ; Vocal Cord Paralysis