Protein-losing enteropathy is a rare complication of autoimmune diseases. We report a case of primary Sjogren's syndrome with protein-losing enteropathy in 50-year-old female who complained of generalized edema. Protein-losing enteropathy of the small intestine was demonstrated by Tc-99m labeled albumin abdominal scintigraphy. Duodenal biopsies showed chronic inflammatory cell infiltration without lymphangiectasis or vasculitis. The patient received oral prednisolone (60 mg/day) for 4 weeks with subsequent clinical improvement. Primary Sjogren's syndrome or other autoimmune diseases should be considered in cases of protein-losing enteropathy.
Autoimmune Diseases ; Biopsy ; Edema ; Female ; Humans ; Intestine, Small ; Lymphangiectasis ; Middle Aged ; Prednisolone ; Protein-Losing Enteropathies* ; Radionuclide Imaging ; Sjogren's Syndrome* ; Vasculitis

BACKGROUND/AIMS: We investigated the electromyography (EMG) findings and demographic, clinical, and laboratory features that may predict the development of malignancy in patients with idiopathic inflammatory myopathy (IIM). METHODS: In total, 61 patients, 36 with dermatomyositis and 25 with polymyositis, were included. Patients were divided into those with and without malignancies, and comparisons were made between the groups in terms of their demographic, clinical, laboratory, and EMG findings. RESULTS: The frequencies of malignancies associated with dermatomyositis and polymyositis were 22% and 8%, respectively. Patients with malignancies showed a significantly higher incidence of dysphagia (odds ratio [OR], 21.50; 95% confidence interval [CI], 3.84 to 120.49), absence of interstitial lung disease (ILD; OR, 0.12; 95% CI, 0.01 to 0.98), and complex repetitive discharge (CRD) on the EMG (OR, 26.25; 95% CI, 2.67 to 258.52), versus those without. After adjustment for age, dysphagia and CRD remained significant, while ILD showed a trend for a difference but was not statistically significant. Multivariate analysis revealed that the CRD conferred an OR of 25.99 (95% CI, 1.27 to 531.86) for malignancy. When the frequency of malignancy was analyzed according to the number of risk factors, patients with three risk factors showed a significantly higher incidence of malignancy, versus those with fewer than two (p = 0.014). CONCLUSIONS: We demonstrated for the first time that CRD on the EMG was an additional independent risk factor for malignancy in IIM. Further studies on a larger scale are needed to confirm the importance of CRD as a risk factor for malignancy in IIM.
Action Potentials ; Adult ; Aged ; Dermatomyositis/complications/*diagnosis/physiopathology ; *Electromyography ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Muscle, Skeletal/*innervation/pathology ; Neoplasms/*etiology ; Odds Ratio ; Polymyositis/complications/*diagnosis/physiopathology ; Predictive Value of Tests ; Retrospective Studies ; Risk Factors

A 73-year-old man with a history of hypertension and ascending aortic dissection was hospitalized for aggravated abdominal pain and general ache for 3 months. Follow-up CT showed aggravated abdominal aortic hematoma with aneurysm, atherosclerotic periaortitis and bilateral hydronephrosis. An initial laboratory finding showed elevated levels of inflammatory markers and renal dysfunction. Positron emission tomography-CT showed an increased standardized uptake values level in the aortic arch, descending thoracic aorta, major branch, abdominal aorta, and common iliac artery. For bilateral hydronephrosis, a double J catheter insertion was performed. Tissue specimens obtained from previous surgery on the aorta indicated the infiltration of lympho-plasma cells without granuloma formation in the aortic wall. After a combined therapy of high dose steroid therapy with azathioprine, the patient's initial complaints of abdominal pain, weakness and azotemia improved. This case was diagnosed as chronic periaortitis based on aortic inflammation at biopsy, which was complicated with retroperitoneal fibrosis and ureteric obstruction.
Abdominal Pain ; Aneurysm ; Aorta ; Aorta, Abdominal ; Aorta, Thoracic ; Azathioprine ; Azotemia ; Biopsy ; Catheters ; Electrons ; Follow-Up Studies ; Granuloma ; Hematoma ; Hydronephrosis ; Hypertension ; Iliac Artery ; Inflammation ; Retroperitoneal Fibrosis ; Ureter

The salient features of Sweet syndrome, also known as acute febrile neutrophilic dermatosis, are pyrexia, elevated neutrophil count, painful red papules, nodules, plaques, and an infiltrate that consists mainly of mature neutrophils diffusely distributed in the upper dermis. In addition to idiopathic forms of the syndrome, malignancies, inflammatory bowel disease, infections, pregnancy, and drugs have been associated with Sweet syndrome. Disseminated gonococcal infection (DGI) results from bacteremic dissemination of Neisseria gonorrhoeae. The most common presentation of DGI is arthritis-dermatitis syndrome. About 75% of DGI patients display a characteristic dermatitis consisting of discrete papules and pustules, often with a hemorrhagic component. We report a case of DGI presenting as Sweet syndrome.
Dermatitis ; Dermis ; Fever ; Humans ; Inflammatory Bowel Diseases ; Neisseria gonorrhoeae ; Neutrophils ; Pregnancy ; Sweet Syndrome

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

OBJECTIVE: To investigate the clinical characteristics of relapsing polychondritis (RP) in Korea. METHODS: We retrospectively reviewed 16 patients with RP who were diagnosed at Seoul National University Hospital and Gyeongsang National University Hospital in the period from July 1987 to August 2003. RESULTS: There were 9 males and 7 females, the mean age was 45.4+/-12.8 years. The frequency of clinical manifestation was as follows; auricular chondritis (81.3%), nasal chondritis (68.8%), ocular inflammation (56.3%), respiratory tract involvement (56.3%), arthritis (50%) and audio-vestibular damage (25%). Westergren erythrocyte sedimentation rate (ESR) was elevated in 53.3% and antinuclear antibody (ANA) was positive in 20% of study patients. Rheumatoid factor was negative in all cases. CONCLUSION: Clinical manifestations were comparable to the reports of Western countries. However, the frequency of arthritis was lower in Korean patients with RP than Western reports.
Antibodies, Antinuclear ; Arthritis ; Blood Sedimentation ; Female ; Humans ; Inflammation ; Korea* ; Male ; Polychondritis, Relapsing* ; Respiratory System ; Retrospective Studies ; Rheumatoid Factor ; Seoul

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*

OBJECTIVE: This study sought to investigate independent predictive factors for subclinical atherosclerosis in Korean patients with rheumatoid arthritis (RA). METHODS: We used high-resolution B-mode ultrasonography to measure the carotid artery intima-media thickness (IMT) and carotid plaque in 367 patients with RA. Detailed information on the demographic characteristics, cardiovascular (CV) risk factors, and RA disease characteristics were collected on all subjects. The relationship of the carotid artery IMT and carotid plaque to relevant clinical and laboratory variables were examined. RESULTS: Old age and male sex had the most significant association with increased IMT and presence of plaque than other factors. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and mKHAQ (Korean version of modified health assessment questionnaire) were significantly associated with both increased IMT and presence of plaque after univariate analysis adjusting for age and sex. A multivariable logistic regression analysis revealed that ESR and TJC68 were independent factors associated with the presence of plaque (p<0.001 and p=0.019, respectively). There was a significant linear correlation between the number of plaques and ESR (p<0.001 and R2=0.07). CONCLUSION: Our results indicated that markers of systemic inflammation contributed significantly to subclinical atherosclerosis in patients with RA. We emphasize the need for aggressive control of RA disease activity in patients who persistently demonstrate highly elevated ESR levels.
Arthritis, Rheumatoid ; Atherosclerosis ; Blood Sedimentation ; C-Reactive Protein ; Cardiovascular Diseases ; Carotid Arteries ; Cohort Studies ; Humans ; Inflammation ; Logistic Models ; Male ; Risk Factors

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

Distal renal tubular acidosis (RTA) is characterized by a decreased net H+ secretion in the collecting tubules, which results in a failure of urine acidification and results in metabolic acidosis, hypokalemia, and nephrocalcinosis. The acquired form of distal RTA is associated with tubulointerstitial involvement of immune-mediated disorders such as Sjogren's syndrome and systemic lupus erythematosus (SLE). Only a few case reports have indicated that distal RTA precedes SLE by months to years. We present a 39-year-old woman who had manifestations of distal RTA for 21 years before the development of overt symptoms of SLE.
Acidosis ; Acidosis, Renal Tubular ; Adult ; Female ; Humans ; Hypokalemia ; Lupus Erythematosus, Systemic ; Nephrocalcinosis ; Sjogren's Syndrome