Myasthenia gravis(MG) is an autoimmune disorder characterized by the presence of anti-acetylcholine receptor antibody and weakness of voluntary muscles. The pathogenesis of MG is decreased numbers of acetylcholine receptors at postsynaptic membranes of neuromuscular junctions. It has been reported that MG often coexists with other autoimmune disorders. This is a case report of systemic lupus erythematosus coexisting with MG in a 23 year old female patient presenting with dysarthria, dysphagia, and limb weakness. We report the case with relevant literature review.
Deglutition Disorders ; Dysarthria ; Extremities ; Female ; Humans ; Lupus Erythematosus, Systemic* ; Membranes ; Muscle, Skeletal ; Myasthenia Gravis* ; Neuromuscular Junction ; Receptors, Cholinergic ; Young Adult

PURPOSE: To evaluate the usefulness of transperineal sonography of the prostate in patients with previousabdominoperineal resection. MATERIALS AND METHODS: Transperineal prostatic sonograms were obtained from ninepatients with abdominoperineal resection. The shortest distance between the probe and the prostate gland, itsvolume, and its internal and external structures were evaluated for image quality, shape, border, and the degreeof distortion in internal echo. Because the number of patients was small, statistical analysis was not performed. RESULTS: Transperineal sonography of the prostate in eight patient was obtained at ease, except that one of ninepatients was not able to continue the procedure because of severe tenderness of the perineum and poor cooperation.Acceptable images of the prostate gland and their adjacent structures were obtained in one patient with less than1cm between the probe and prastate gland, three of four with between 1cm and 1.5cm, and one of three with morethan 1.5cm. CONCLUSION: In patients with previous abdominoperineal resection, transperineal sonography of theprostate may be a good imaging modality.
Humans ; Perineum ; Prostate*

OBJECTIVE: To investigate the autoantibody profile and its clinical association in patients with systemic lupus erythematosus. METHODS: The frequency and clinical correlation of autoantibodies were studied in 73 patients with systemic lupus erythematosus who have been followed in Seoul National University Hospital. Double immunodiffusion, ELISA and immunoblot were used for the detection of autoantibodies. RESULTS: The frequency of each autoantibody measured by double immunodif fusion was as follows; anti-Ro 53.4%, anti-La 11.0%, anti-Sm 20.5%, anti-U1 RNP 20.5%. The frequency of each autoantibody by ELISA was as follows; anti-Ro 69.9%, anti-La 27.4%, anti-Sm 54.8%, anti-Ul RNP 68.5%, anti-dsDNA 72.6%, anti-cardiolipin 47.2% (IgG 43.1?0, igM 15. 3%). The frequency of each autoantibody by immunoblot was as follows; anti-Ro 15.1?0, anti-La 42. 5%, anti-Sm 46. 6%, anti-U1 RNP 42. 5%. anti-ribosomal P(P0) 27.4%. Anti-Ro was associated with decreased frequency of nephrotic syndrome. Anti-U1 RNP was associated with increased frequency of malar rash, Raynaud phenomenon and decreased frequency of nephritis. Patients with both anti-Ro and anti La had more frequent serositis than those with anti-l~o only. Patients with both anti-Sm and anti-U1 RNP had less frequent thrombocytopenia than those with anti-U1 RNP only. And patients with anti-Sm and anti-dsDNA had more frequent arthritis than those with only one of both antibodies. There was a positive correlation of autoantibody titers between anti-Ro and anti-La, anti-Sm and anti-U1 RNP, anti-dsDNA and anti-cardiolipin(IgG). Taking the result of immunoblot as a standard, both of double immunodiffusion and ELISA showed low sensitivity but high specficity for anti La. As for anti-Sm and anti-U1 RNP, double immunodiffusion showed low sensitivity but high specificity, whereas ELISA showed high sensitivity but low specificity. CONCLUSIONS: In our study, some autoantibodies (anti-Ro, anti-U1 RNP) were associated with certain clinical manifestations while others not. Immunoblot being used as a standard method, ELISA showed higher sensitivity but lower specificity for anti-La, anti-Sm and anti-U1 RNP compared with immunodiffusion. It is recommended that in interpretating the laboratory findings of these autoantibodies these parameters of each method should be considered.
Antibodies ; Arthritis ; Autoantibodies ; Blotting, Western* ; Enzyme-Linked Immunosorbent Assay* ; Exanthema ; Humans ; Immunodiffusion* ; Immunoglobulin M ; Lupus Erythematosus, Systemic ; Nephritis ; Nephrotic Syndrome ; Raynaud Disease ; Sensitivity and Specificity ; Seoul ; Serositis ; Thrombocytopenia

Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis involving skin, gut, and kidney associated with arthralgia, arthritis and characterized by immunoglobulin A (IgA)-dominant immune deposits in target organs. This vasculitis is common in children and rarely reported to be associated with other autoimmune disease such as rheumatoid arthritis, autoimmune thyroiditis. We report the case of 72 year-old man who developed adult-onset HSP and also had rheumatoid arthritis and chronic lymphocytic thyroiditis. This is the first report in South Korea.
Aged ; Arthralgia ; Arthritis* ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Child ; Hashimoto Disease* ; Humans ; Immunoglobulin A ; Kidney ; Korea ; Purpura, Schoenlein-Henoch* ; Skin ; Thyroiditis ; Thyroiditis, Autoimmune ; Vasculitis

We describe a case of a 54-yr-old AL amyloidosis patient who developed femoral compressive neuropathy due to iliopsoas pseudohypertrophy. The patient, who presented with end stage renal disease, was referred to our clinic because of lower extremity weakness and polyarthritis. Finally, he was diagnosed as having kappa-AL amyloidosis, complicated by femoral compressive neuropathy, hypertrophic amyloid myopathy, amyloid arthropathy, carpal tunnel syndrome, and end stage renal disease. Femoral compressive neuropathy has never been reported in association with amyloid induced iliopsoas hypertrophic myopathy. This report expands the clinical spectrum of AL amyloidosis.
Amyloidosis/*pathology ; Fatal Outcome ; Femoral Neuropathy/*pathology ; Humans ; Hypertrophy ; Kidney Failure, Chronic/pathology ; Male ; Middle Aged ; Nerve Compression Syndromes/*pathology ; Psoas Muscles/pathology

Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.
Scleroderma, Systemic/*complications/enzymology/*immunology ; RNA Polymerase III/*immunology ; RNA Polymerase I/*immunology ; Korea ; Kidney Failure, Acute/*complications/enzymology/*immunology ; Humans ; Female ; Critical Care ; Autoantibodies/*blood ; Aged

Systemic sclerosis is a rare, autoimmune, connective tissue disorder which involves skin and other internal organs. However, central nervous system involvement including seizure, is a very rare event. This 29-year-old woman experienced repeated episodes of typical generalized tonic clonic seizure. T2 weighted and flare images of magnetic resonance image showed a mass-like high signal intensity lesion in the left temporal lobe. Atypical spikes and waves were present in a eclectroencephalogram. Her seizure episodes were controlled with anticonvulsants, corticosteroids and immunosuppresants. We report a rare case of systemic sclerosis which showed central nervous system involvement.
Adrenal Cortex Hormones ; Adult ; Anticonvulsants ; Central Nervous System ; Connective Tissue ; Female ; Humans ; Magnetic Resonance Imaging ; Scleroderma, Diffuse* ; Scleroderma, Systemic ; Seizures* ; Skin ; Temporal Lobe

Dendritic cells (DCs) play a key role in activating the immune response against invading pathogens as well as dying cells or tumors. Although the immune response can be initiated by the phagocytic activity by DCs, the molecular mechanism involved in this process has not been fully investigated. Trp-Lys-Tyr-Met-Val-Met-NH2 (WKYMVM) stimulates the activation of phospholipase D (PLD) via Ca2+ increase and protein kinase C activation in mouse DC cell line, DC2.4. WKYMVM stimulates the phagocytic activity, which is inhibited in the presence of N-butanol but not t-butanol in DC2.4 cells. Furthermore, the addition of phosphatidic acid, an enzymatic product of PLD activity, enhanced the phagocytic activity in DC2.4 cells. Since at least two of formyl peptide receptor (FPR) family (FPR1 and FPR2) are expressed in DC2.4 as well as in mouse bone marrow-derived dendritic cells, this study suggests that the activation of FPR family by WKYMVM stimulates the PLD activity resulting in phagocytic activity in DC2.4 cells.
1-Butanol/pharmacology ; Animals ; Bone Marrow Cells/cytology/metabolism ; Calcium Signaling/*drug effects ; Cell Death/immunology ; Cell Line ; Communicable Diseases/immunology ; Dendritic Cells/immunology/*metabolism ; Mice ; Neoplasms/immunology ; Oligopeptides/*pharmacology ; Phagocytosis/*drug effects ; Phosphatidic Acids/pharmacology ; Phospholipase D/*metabolism ; Receptors, Formyl Peptide/*metabolism ; Research Support, Non-U.S. Gov't ; tert-Butyl Alcohol/pharmacology

PURPOSE: Diabetic foot ulcers are one of the most important complications of patients with diabetes for their quality of life. Yet the data on factors that affect the treatment outcome and the guidelines for a multidisciplinary approach are limited. The purpose of this study was to assess the clinical characteristics that affect healing of diabetic foot ulcers, and especially when this is associated with peripheral arterial disease (PAD). METHOD: We retrospectively reviewed the clinicopathologic data of 112 patients who were admitted for the treatment of diabetic foot ulcers from May 2004 to December 2007 at Bundang Seoul National University Hospital. The patient's demography and co-morbidities, the laboratory and radiological details, the surgical data and the treatment outcomes were evaluated and analyzed according to the presence of PAD. RESULT: The mean age of the patients was 66 years old and the male to female ratio was 2.3:1. Except for simple dressing of the ulcers, skin graft, amputation or revascularization were performed for 77 patients. Ulcers healed in 74 patients (66.1%) and recurrence was observed in 42 patients (37.5%). Major amputation was performed on 11 patients (9.8%). The non-healing group had a higher incidence of male gender (P=0.049), end stage renal disease (P=0.038), coronary arterial disease (P=0.018), the presence of PAD (P=0.034) and a higher level of cholesterol (P=0.011) and triglyceride (P=0.039). Patients with PAD had a lower ankle-brachial index (P<0.001) and a higher rate of undergoing revascularization (P<0.001), overall amputation (P=0.003), non-healing (P=0.034) and recurrence (P<0.001). After revascularization, the rate of major amputation was not reduced (P=0.915). CONCLUSION: The risk of non-healing, overall amputation and recurrence is increased in the presence of PAD. Evaluating the PAD status and multidisciplinary treatment strategies are needed to treat these patients with diabetic foot ulcer.
Amputation ; Ankle Brachial Index ; Bandages ; Cholesterol ; Demography ; Diabetic Foot ; Female ; Humans ; Incidence ; Kidney Failure, Chronic ; Male ; Peripheral Arterial Disease ; Quality of Life ; Recurrence ; Retrospective Studies ; Skin Ulcer ; Transplants ; Treatment Outcome ; Ulcer

Relapsing polychondritis is a rare autoimmune disease of unknown etiology characterized by recurrent inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. Relapsing polychondritis may affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. Several cardiovascular complications have been reported in patients with relapsing polychondritis. The most common findings of cardiovascular manifestations are aortic or mitral regurgitation and aortic aneurysm. Aortitis is a very rare complication of relapsing polychondritis. We report a case of 48-year-old man who had relapsing polychondritis with aortitis mimicking Takayasu's arteritis.
Aorta ; Aortic Aneurysm ; Aortitis ; Autoimmune Diseases ; Ear ; Heart ; Humans ; Inflammation ; Joints ; Middle Aged ; Mitral Valve Insufficiency ; Nose ; Polychondritis, Relapsing* ; Respiratory System ; Skin ; Takayasu Arteritis* ; Vasculitis*