No abstract available.
Hip ; Hip Joint ; Osteoma, Osteoid

No abstract available.
Amyloidosis

Churg-Strauss syndrome is a rare systemic disorder characterized by asthma, eosionphilia and necrotizing vasculitis affecting small-to-medium-sized vessels. Although it is frequently associated with gastrointestinal mucosal lesions, recurrent bowel perforation is rare and potentially life threatening. We report a case of a 66-year-old man with Churg-Strauss syndrome, who presented with recurrent small bowel perforation. He was admitted with abdominal pain developed previous night, who had a previous small bowel perforation history treated with laparoscopic closure 5 months ago. Laboratory data showed remarkable eosinophilia. Physical examination indicated positive signs of peritoneal irritation in the entire abdomen, and abdominal computed tomography scanning showed edematous small bowel with intra-abdominal free air, suggesting intestinal perforation. He underwent laparoscopic small bowel closure and was treated with steroid.
Abdomen ; Abdominal Pain ; Aged ; Asthma ; Churg-Strauss Syndrome ; Eosinophilia ; Humans ; Intestinal Perforation ; Physical Examination ; Vasculitis

Microscopic polyangiitis (MPA) is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. The kidney is the most often affected organ in the majority of patients with MPA, and renal manifestations are usually the first symptoms. Glomerular capillaries are affected most often, resulting in necrotizing glomerulonephritis, usually in a crescent formation, with no or few immune deposits able to be demonstrated at the sites of vasculitis and glomerulonephritis. We report a case of microscopic polyangiitis in both legs with pitting edema in a 50-year-old female. Laboratory findings showed hematuria, proteinuria, and a positive peripheral antineutrophil cytoplasmic antibody. A renal biopsy revealed pauci-immune splitting and necrotizing capillary loop walls necrotizing vasculitis and membranoproliferative glomerulonephritis (MPGN). With a diagnosis of MPA, she has been managed with high dose steroid and cyclophosphamide. To our knowledge, this is the first reported case of MPA with MPGN.
Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Capillaries ; Cyclophosphamide ; Edema ; Female ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Glycosaminoglycans ; Hematuria ; Humans ; Inflammation ; Kidney ; Leg ; Microscopic Polyangiitis ; Middle Aged ; Proteinuria ; Vasculitis

Renal involvement in systemic lupus erythematosus (SLE) is a typical manifestation of the disease. The occurrence of non-lupus nephritis, especially IgA nephropathy (IgAN), in patients with SLE has rarely been reported. We describe the case of a 21-year-old woman who was diagnosed with IgAN and subacute necrotizing lymphadenitis, and her renal lesion biopsy was typical of lupus nephritis (ISN/RPS Class III). Although IgAN and lupus nephritis share some common physiopathological characteristics, their laboratory, histopathologic findings, and the extra-renal clinical manifestations are different and support a different pathogenesis. Our case highlights the importance of a renal biopsy in patients with lupus and urinary alterations despite underlying IgAN. A correct diagnosis would permit the most appropriate immunosuppressive treatments to be considered.
Biopsy ; Female ; Glomerulonephritis, IGA ; Humans ; Immunoglobulin A ; Kidney ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Lymphadenitis ; Nephritis ; Young Adult

Renal involvement is one of the extra-articular manifestations found in patients with rheumatoid arthritis (RA). Membranous glomerulonephopathy, membranoproliferative glomeruonophritis, secondary amyloidosis, and focal segmental glomerulosclerosis are reported as pathologic diagnoses of renal involvement. However, reports of renal involvement in patients with RA and antineutrophil cytoplasmic autoantibody (ANCA)-associated pauci-immune glomerulonephritis are rare. Recently, we experienced two patients with RA who developed azotemia and were finally diagnosed with ANCA-associated pauci-immune glomerulonephritis. Because of the rarity of these cases, we report two cases in patients with RA with a literature review.
Amyloidosis ; Arthritis, Rheumatoid ; Azotemia ; Cytoplasm ; Glomerulonephritis ; Glomerulosclerosis, Focal Segmental ; Humans

Cardiac involvement is an important cause of mortality in patients with Churg-Strauss syndrome. The typical cardiac presentation of Churg-Strauss syndrome includes pericarditis, myocarditis, and cardiomyopathy. Endomyocardial fibrosis has rarely been described in patients with Churg-Strauss syndrome. We experienced a patient with Churg-Strauss syndrome who exhibited exertional dyspnea and endomyocardial fibrosis visualized as delayed enhancement on cardiac magnetic resonance imaging (MRI). After glucocorticoid treatment, the patient's symptom resolved, and the eosinophil count decreased to normal. Nine months later, the delayed-enhanced lesion on the cardiac MRI nearly disappeared. Here, we report a case of endomyocardial fibrosis in a patient with Churg-Strauss syndrome with a literature review.
Cardiomyopathies ; Churg-Strauss Syndrome ; Dyspnea ; Endomyocardial Fibrosis ; Eosinophils ; Humans ; Magnetic Resonance Imaging ; Myocarditis ; Pericarditis

Cogan's syndrome can accompany a variety of systemic vasculitides including aortitis. A 45-year-old woman with a history of typical Cogan's syndrome presented with orthopnea and exertional dyspnea. Echocardiography demonstrated severe aortic valve insufficiency. Computed tomographic angiography demonstrated active vasculitis affecting the ascending and descending aortas and also showed stenosis of the left subclavian artery, both renal arteries, the celiac axis, the superior mesenteric artery, and the right common iliac artery. She received high dose corticosteroid and then underwent an aortic valve replacement. This is the first case of Cogan's syndrome with aortitis in Korea.
Angiography ; Aorta, Thoracic ; Aortic Valve ; Aortic Valve Insufficiency ; Aortitis ; Arthritis ; Axis, Cervical Vertebra ; Cogan Syndrome ; Constriction, Pathologic ; Dyspnea ; Echocardiography ; Female ; Humans ; Iliac Artery ; Korea ; Mesenteric Artery, Superior ; Middle Aged ; Renal Artery ; Subclavian Artery ; Systemic Vasculitis ; Vasculitis

Acquired hemophilia is a rare disease caused by an autoimmune reaction to coagulation factor VIII, The mortality rate of this disease is very high (8~22%). Clinical manifestations are different from congenital hemophilia. Various diseases are associated with acquired hemophilia, including autoimmune diseases such as systemic lupus erythematosus or rheumatoid arthritis (RA), tumors, inflammatory bowel disease, psoriasis, asthma, diabetes, acute hepatitis B or C, and drug reactions. However, the underlying cause is unknown in approximately 50% of cases. A few cases of acquired hemophilia with RA have been published. However, no cases have been reported in Korea. We had a patient with longstanding RA and acquired hemophilia who was suffering from upper and lower extremity purpura with a deep intramuscular hematoma. The patient was successfully treated using cyclophosphamide combined with steroid.
Arthritis, Rheumatoid ; Asthma ; Autoimmune Diseases ; Cyclophosphamide ; Factor VIII ; Hematoma ; Hemophilia A ; Hepatitis B ; Humans ; Inflammatory Bowel Diseases ; Korea ; Lower Extremity ; Lupus Erythematosus, Systemic ; Psoriasis ; Purpura ; Rare Diseases ; Stress, Psychological

It is occasionally difficult to distinguish Wegener's granulomatosis (WG) from other diseases including malignancy, tuberculosis, and various types of vasculitis because of the overlapping symptoms and signs. We report on a patient with NK/T cell lymphoma who was treated with a limited form of WG. At his first visit, he presented with left foot drop and recurrent nasal swelling. Necrosis and massive infiltration of inflammatory cells were identified on a nasal tissue biopsy. Sural nerve biopsy findings also showed infiltration of inflammatory cells in both the endoneurium and perivascular area; thus, a diagnosis of a limited form of WG was made. After combination therapy with a glucocorticoid and oral cyclophosphamide was initiated, his condition completely recovered without recurrence for the next 2 years. However, he visited the hospital again for recurrence of nasal swelling. Repeated biopsy of nasal tissues, combined with an immunophenotypic analysis revealed NK/T cell lymphoma. The possibility of NK/T lymphoma should be considered when evaluating a limited type of WG, which shows atypical findings on biopsy as well as recurrent deterioration, as a suboptimal dose of immunosuppressive therapy may mask its expression and lead to a poor prognosis.
Biopsy ; Cyclophosphamide ; Foot ; Humans ; Lymphoma ; Masks ; Necrosis ; Organic Chemicals ; Peripheral Nerves ; Prognosis ; Recurrence ; Sural Nerve ; Tuberculosis ; Vasculitis ; Wegener Granulomatosis