No abstract availble.
Autoimmune Diseases/*diagnosis ; Bile Duct Diseases/*diagnosis ; Cholangitis, Sclerosing/*diagnosis ; Diagnosis, Differential ; Female ; Humans ; Male ; Pancreatitis/*diagnosis/immunology

Autoimmune Diseases ; diagnosis ; immunology ; pathology ; therapy ; Child ; China ; Gastroenterology ; Hepatitis B ; diagnosis ; pathology ; therapy ; Humans ; Infant

Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.
Aged ; Autoimmune Diseases/diagnosis ; Female ; Granuloma, Plasma Cell/*diagnosis ; Humans ; Immunoglobulin G/*immunology ; Pancreatitis/diagnosis/immunology ; Sclerosis ; Urethral Diseases/*diagnosis/immunology

OBJECTIVETo assess the seroprevalence and diagnostic value of aquaporin-4 antibody (AQP4-Ab) in patients with inflammatory central nervous system demyelinating diseases.

METHODSSeventy-two patients with neuromyelitis optica (NMO), 68 with multiple sclerosis (MS), 4 with optic neuritis (ON), and 41 with transverse myelitis (TM) were included in this study. The TM group comprised 19 patients with non-longitudinally extensive transverse myelitis (nLETM), 14 with monophasic longitudinally extensive transverse myelitis (mLETM), and 8 with recurrent longitudinally extensive transverse myelitis (rLETM). The serum levels of AQP4-Ab was detected by indirect immunofluorence assay in these patients.

RESULTSAQP4-Ab was detected in 72.2% (52/72) patients with NMO, 5.9% (4/68) patients with MS, 25.0% (1/4) patients with ON, and 17.1% (7/41) patients with TM, showing a significant difference in the positivity between NMO and MS groups (P<0.01). AQP4-Ab seropositivity rate was 5.3% (1/19) in nLETM patients, 62.5% (5/8) in rLETM patients and 7.1% (1/14) in mLETM patients, significantly higher in rLETM than in nLETM (P<0.01) and mLETM groups (P<0.05), but no statistical difference was found between rLETM and NMO groups.

CONCLUSIONSA high seroprevalence of AQP4-Ab is observed in patients with NMO and rLETM, which support the hypothesis that NMO and rLETM belong to NMO spectrum disorders. AQP4-Ab can serve as a useful index for diagnosing NMO and differential diagnosis from MS. More attention and effective immunosuppressive treatments should be given to patients positive for AQP4-Ab.

Aquaporin 4 ; immunology ; Autoantibodies ; blood ; Demyelinating Autoimmune Diseases, CNS ; diagnosis ; immunology ; Female ; Humans ; Male ; Multiple Sclerosis ; diagnosis ; immunology ; Neuromyelitis Optica ; diagnosis ; immunology ; Seroepidemiologic Studies

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

IgG4-related disease (IgG4-RD) is a novel and rare autoimmune disease entity. Elevated serum IgG4 level is strongly suggestive of IgG4-RD. But it is still unknown whether serum IgG4 elevation commonly occurs in other autoimmune diseases. In this study, the serum IgG4 levels were detected by an established enzyme-linked immunosorbent assay (ELISA) in a variety of autoimmune diseases including systemic lupus erythematosus (SLE), Sjogren's syndrome (SS), polymyositis or dermatomyositis (PM/DM) and IgG4-RD. To evaluate the reliability of this ELISA system, some of our samples were sent to a lab in Kanazawa Medical University, Japan, and detected by using the nephelometric assay. The results showed that our findings were consistent with theirs. Moreover, it was found that the serum IgG4 levels were 0.23±0.16 g/L in 53 healthy controls, 0.16±0.15 g/L in 103 SLE patients, 0.22±0.18 g/L in 41 SS patients and 0.40±0.32 g/L in 21 PM/DM patients. No significant difference in the serum IgG4 level was observed among these groups (P>0.05). The serum IgG4 levels of two cases of IgG4-RD were 1.63 and 4.65 g/L respectively, and both decreased markedly after treatment with glucocorticoids. These data indicated that this established ELISA system can be used for detecting serum IgG4 levels. Elevated serum IgG4 levels help diagnose IgG4-RD and evaluate the curative effect of this condition rather than other autoimmune diseases.
Autoimmune Diseases ; blood ; diagnosis ; immunology ; Enzyme-Linked Immunosorbent Assay ; methods ; Humans ; Immunoglobulin G ; blood

Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.
Appendiceal Neoplasms/*diagnosis ; Appendicitis/*diagnosis ; Appendix/immunology/*pathology ; Autoimmune Diseases/*diagnosis/immunology ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*immunology ; Male ; Middle Aged ; Neoplasms

Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.
Adult ; Antibodies, Anti-Idiotypic/immunology ; Autoimmune Diseases/*diagnosis/immunology ; Humans ; Immunoglobulin G/*immunology ; Intestine, Small/*pathology/radiography ; Male ; Multidetector Computed Tomography/*methods ; Sclerosis/diagnosis/immunology

OBJECTIVETo investigate the relation between insulin resistance and glutamic acid decarboxylase antibody (GAD-Ab) titers in latent autoimmune diabetes in adults (LADA).

METHODSThe patients with phenotypic type 2 diabetes were screened for GAD-Ab positivity, and the 141 positive patients were divided into two subgroups according to the GAD-Ab titer, namely the high-titer group (LADA-1 subtype) and low-titer group (LADA-2 subgroup). The clinical features and insulin resistance were compared between the two groups. Insulin resistance was calculated by HOMA 2 software, and GAD-Ab and C peptide were determined with radioligand and radioimmune assay, respectively.

RESULTSCompared with low-titer LADA patients, the patients with high titers had younger age of onset, lower BMI, higher HbA1c level, and worse fasting and postprandial C peptide levels. The insulin resistance index by HOMA 2 was significantly lower in LADA-1 group than in LADA-2 group (1.6-/+1.1 vs 2.1-/+1.1, P=0.001). The HOMA2-IR index showed a negative correlation to GAD-Ab titer.

CONCLUSIONThe degree of insulin resistance is correlated to GAD-Ab titers in LADA, and low titer patients have higher insulin resistance level.

Adult ; Aged ; Autoantibodies ; blood ; Autoimmune Diseases ; diagnosis ; immunology ; Diabetes Mellitus, Type 2 ; diagnosis ; immunology ; Female ; Glutamate Decarboxylase ; immunology ; Humans ; Insulin Resistance ; Islets of Langerhans ; immunology ; physiology ; Male ; Middle Aged

BACKGROUND: The indirect basophil activation test using flow cytometry is a promising tool for autoimmune urticaria diagnosis. We aimed to identify better donor basophils (from atopic vs. non-atopic donors and interleukin-3 primed vs. unprimed basophils) and improve basophil identification and activation markers (eotaxin CC chemokine receptor-3 [CCR3] vs. CD123 and CD63 vs. CD203c). METHODS: Donor basophils were obtained from non-atopic and atopic group O donors. Positive control sera were artificially prepared to simulate autoimmune urticaria patients' sera. Patient sera were obtained from nine children with chronic urticaria. Assay sensitivity was compared among each variation by using positive control sera (n=21), applying cutoff values defined from negative control sera (n=20). RESULTS: For basophil identification, a combination of CCR3 and CD123 markers revealed a higher correlation with automated complete blood count (r=0.530) compared with that observed using CD123 (r=0.498) or CCR3 alone (r=0.195). Three activation markers on the atopic donor basophils attained 100% assay sensitivity: CD203c on unprimed basophils, CD63+CD203+ or CD63 alone on primed basophils; however, these markers on the non-atopic donor basophils attained lower assay sensitivity. CONCLUSIONS: For basophil identification markers, a combination of CD123 and CCR3 is recommended, while CD123 alone may be used as an alternative. Donor basophils should be obtained from an atopic donor. For basophil activation markers, either CD203c alone on unprimed basophils or CD203c and CD63 on primed basophils are recommended, while CD63 alone on primed basophils may be used as an alternative.
Autoimmune Diseases/blood/*diagnosis/immunology ; Basophils/*immunology/metabolism ; Biomarkers/blood ; Child ; Flow Cytometry ; Humans ; Interleukin-3 Receptor alpha Subunit/blood ; Male ; Receptors, CCR3/blood ; Urticaria/blood/*diagnosis/immunology