Systemic lupus erythematosus (SLE) is a prototype for multi-system, autoimmune diseases of unknown etiology, characterized by the production of autoantibodies. SLE can involve any organ system of the body with constitutional symptoms, including musculoskeletal, skin, renal, neuropsychiatric, cardiovascular, respiratory and gastrointestinal systems. These wide spectra of disease manifestations have made disease classification difficult. American College of Rheumatology (ACR) proposed classification criteria for SLE for research purpose in 1982, which had been widely used for research purpose and not for diagnosis. In 1997, these criteria were updated with further recognition of antiphospholipid antibodies, but not validated. But ACR criteria didn't still meet the necessity for earlier diagnosis of SLE. In order to improve clinical relevance and incorporate new knowledge to the field of lupus immunology, the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC), an international lupus expert group dedicated to clinical research on lupus, revised the ACR systemic lupus classification criteria in 2012. The new 2012 SLICC criteria were validated using a large set of patient scenarios rated by experts. The history and diagnostic utility of SLE criteria are covered in this review.
Allergy and Immunology ; Antibodies, Antiphospholipid ; Autoantibodies ; Autoimmune Diseases ; Classification* ; Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Rheumatology ; Skin

Urticaria is a common cutaneous disease characterized by recurrent and transient wheals and pruritus, sometimes accompanied angioedema. The classification of urticaria is based on the duration of the disease and whether extrinsic triggers are identified or not. Acute urticaria is usually occurred by specific causes, such as drug, food, and infection, etc. Therefore, acute urticaria can be remitted within 6 weeks just by avoiding the exposure to the causes. However, chronic urticaria defined as repeatedly occurred itchy wheals and/or angioedema for at least 6 weeks, has a significant effect on patients' quality of life. Chronic inducible urticaria can be triggered by various physical stimuli including dermographism, delayed pressure, cold, heat, cholinergic stimuli, sunlight, and exercise. Chronic spontaneous urticaria (CSU) is diagnosed when no specific extrinsic cause is identified in the patients. CSU due to autoimmune mechanism accounts for 30–50%, autologous serum skin test and anti-thyroid autoantibody can be evaluated. However, various physical stimuli, emotional or physical stress, drugs, particularly aspirin and non-steroidal anti-inflammatory drugs can exacerbate urticaria in 30–75% of patients with CSU. Allergic diseases and autoimmune diseases are more common in CSU patients than in general populations. To assess the severity of urticaria and to adjust treatment step, urticaria activity score over 7 days, calculated by the number of wheals and the severity of pruritus, is recommended by recent international guidelines.
Angioedema ; Aspirin ; Autoimmune Diseases ; Classification ; Diagnosis ; Hot Temperature ; Humans ; Pruritus ; Quality of Life ; Skin Tests ; Sunlight ; Urticaria

Smooth muscle antibodies (SMAs) are diagnostic markers for the serological diagnosis of type 1 autoimmune hepatitis. SMA that is restricted to staining of the stomach muscle and blood vessel walls was referred to as "SMA-V". In addition, SMAs are classified into the peritubular (SMA-T) and glomerular (SMA-G) patterns. SMAs are occasionally present in patients with malignancies, but have not yet been reported in thyroid cancer. We came across the first case of SMA positivity in a patient with papillary thyroid carcinoma (PTC). A 31-yr-old male was admitted to our hospital for evaluation of incidentally detected thyroid cancer. He had been diagnosed with PTC based on pathological results following fine-needle aspiration biopsy. The patient underwent total thyroidectomy followed by radio-iodine treatment. The serum levels of AST and ALT were increased before radiotherapy. Tests were conducted for the evaluation of liver disease. SMA was positive at a titer of 1:320, showing positive results for the vessel walls but negative results for the glomerulus and tubules in the kidney (SMA-V pattern). The association of SMA with malignancies and the classification of SMA immunofluorescent subtypes have been previously reported. However, these studies have not clearly established the ability of SMA subtype to predict a specific disease. Therefore, evaluation of an association of SMA pattern with specific diseases in SMA-positive patients may provide additional and useful information for the rapid diagnosis and accurate treatment of patients with autoimmune diseases or malignancies. This case report could serve as a great resource for further studies on SMA.
Antibodies ; Autoimmune Diseases ; Biopsy, Fine-Needle ; Blood Vessels ; Classification ; Diagnosis ; Hepatitis, Autoimmune ; Humans ; Kidney ; Liver Diseases ; Male ; Muscle, Smooth* ; Radiotherapy ; Stomach ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

BACKGROUND/AIMS: There are no pathognomonic features of autoimmune hepatitis (AIH). Its diagnosis requires the exclusion of various other conditions. The aim of this study was to validate indirectly the International Autoimmune Hepatitis Group (IAHG) scoring system in diagnosing AIH. METHODS: Twenty-six patients with Type 1 AIH and female patients with chronic hepatitis B (n=34), chronic hepatitis C (n=25), or toxic hepatitis (n=13) were evaluated according to 9 categories of pretreatment minimum required parameters proposed by IAHG. Aggregate scores of AIH to those of non-AIH groups, which were assessed before and after extracting the proportions of etiologic factors, were also compared and evaluated. RESULTS: While aggregate scores of non-AIH groups, before extracting the proportions of etiologic factors, were 5.2+/-1.8, 5.6+/-1.1, and 7.4+/-1.2 in that order, those of AIH groups were 12.8+/-1.7. These were significantly higher than those of non-AIH groups (p<0.01). All patients in AIH groups and only 1 patient in a non-AIH group showed aggregate scores of more than 10. Aggregate scores after extracting the proportions of etiologic factors were more than 4 in all, except 2, patients. These should have been consistent with 10 if there were no etiologic factors in non-AIH groups. CONCLUSION: The IAHG scoring system might have a relatively excessive importance to the scores of categories excluding distinct etiologies from AIH. It might be difficult to differentiate AIH from chronic liver diseases of indistinct cause based on the IAHG scoring system.
Adult ; Aged ; Autoimmune Diseases/*classification/diagnosis ; English Abstract ; Female ; Hepatitis/*classification/diagnosis/immunology ; Hepatitis B, Chronic/classification/diagnosis ; Hepatitis C, Chronic/classification/diagnosis ; Hepatitis, Toxic/classification/diagnosis ; Human ; Korea ; Male ; Middle Aged

OBJECTIVE: Anti-neutrophil cytoplasmic antibody (ANCA) is an important marker for the diagnosis and the classification of rheumatic diseases and systemic vasculitis. If the autoantibodies that can be stained in the nucleus of neutrophil used for substrate of ANCA test, there's a need for its differential diagnosis from real p-ANCA. This paper focuses on digital image processing technique for the differentiation of p-ANCA without performing additional tests. METHODS: Positive ANCA results which showed mixed fluorescent pattern were transformed into digital image. Using Matlab (MathWorks, U.S.A.), we developed 2D to 3D transformation method and virtual tomography for the interpretation of mixed fluorescent pattern, and compared these results with ANA and anti-DNA test by indirect immunofluorescence (IIF) method using IT-AIT, IT-ANCA, and IT-DNA kit (ImmunoThink, Korea). RESULTS: By applying the 3D transformation method and virtual tomography to the results of ANCA test where combined antibodies exist, we were able to separate each different fluorescent pattern that were difficult to separate by manual reading. CONCLUSION: The new digital image analysis methods developed in this study displace some of the disadvantages of IIF method. Therefore, these methods can easily be applied to complex samples, and can allow rapid and accurate tests for rheumatic diseases and other autoimmune diseases.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic* ; Antibodies, Antinuclear ; Autoantibodies ; Autoimmune Diseases ; Classification ; Diagnosis ; Diagnosis, Differential ; Fluorescent Antibody Technique, Indirect ; Image Processing, Computer-Assisted ; Neutrophils ; Rheumatic Diseases ; Systemic Vasculitis

PURPOSE: Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. METHODS: The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. RESULTS: The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). CONCLUSION: Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.
Adolescent ; Autoimmune Diseases ; Child* ; Classification ; Diagnosis ; DNA ; Edema ; Exanthema ; Female ; Fever ; Humans ; Immunologic Factors ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Male ; Prognosis ; Retrospective Studies

Interstitial lung disease is a generic term for a heterogeneous group of lung disease that primarily affect the interstitium although the disease is not clearly restricted to the interstitium. The majority of interstitial lung diseases represent inflammatory insults to the microscopic anatomic space bounded by the basement membrane of epithelial and endothelial cells, which may occur as slowly developing process and ultimately end up as end-stage honeycomb fibrosis. The currently prevalent classification of interstitial pneumonia with practical utility and easy reproducibility pertaining only to idopathic interstitial pneumonia encompasses several different entities some of which may represent different aspects of the same condition. Honeycomb fibrosis is usually caused by a variety of pulmonary disease including chronic interstitial lung disease. It is important to recognize that usual inter-stitial pneumonia and honeycomb fibrosis are not synonymous. In the era of chemotherapy for malignant tumor, aggressive immunosuppression for autoimmune diseases and transplant recipients and acquired immunodeficiency syndrome, lung disease in the immunocompromised host has been common. Diagnostic lung biopsy becomes increasingly needed because proper treatment of interstitial lung disease relies on correct morphologic diagnosis. This review summarizes the pathologic spectrum of idiopathic interstitial pneumonias together with other inflammatory process with known or suggestive etiologies simulating interstitial pneumonias.
Acquired Immunodeficiency Syndrome ; Autoimmune Diseases ; Basement Membrane ; Biopsy ; Classification ; Diagnosis ; Drug Therapy ; Endothelial Cells ; Fibrosis ; Idiopathic Interstitial Pneumonias ; Immunocompromised Host ; Immunosuppression ; Lung ; Lung Diseases ; Lung Diseases, Interstitial* ; Pathology* ; Pneumonia ; Transplantation

PURPOSE: Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. METHODS: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. RESULTS: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1 (2-18) years old. FANA(95.0%), anti-ds DNA antibody (87.5%), malar rash (80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria (75.0%), nephrotic syndrome (55.0%), and microscopic hematuria alone (15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was 51.8 40.5 months. During 51.8+/-40.5 months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. CONCLUSION: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.
Acute Kidney Injury ; Adult ; Autoimmune Diseases ; Biopsy ; Child* ; Classification ; Cyclophosphamide ; Diagnosis ; DNA ; Exanthema ; Female ; Follow-Up Studies ; gamma-Globulins ; Gonads ; Hematuria ; Humans ; Lupus Nephritis* ; Male ; Mortality ; Nephrotic Syndrome ; Plasma Exchange ; Prednisolone ; Prognosis ; Proteinuria ; Retrospective Studies ; Risk Factors ; Treatment Outcome*