Significance of neuron-specific enolase and antinuclear antibody in the diagnosis of Paraneoplastic neurologic syndrome
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Objective To identify the significance of neuron-specific enolase(NSE) and antinuclear antibody(ANA),erythrocyte sedimentation rate(ESR),C-reactive protein(CRP) in the diagnosis of Paraneoplastic neurologic syndrome. Methods There were forty-five sera from the patients of Paraneoplastic neurologic syndrome and other neurologic system diseases as controls respectively. NSE was detected by the mean of radioimmunity,ANA was detected by indirect immunofluorenscence,ESR was detected by photo-electric scanning,and CRP was detected by turbidimetric immunoassay. Results There were nine patients in the Paraneoplastic neurologic syndrome group whose NSE were elevated. All of these patients had small cell lung cancer. There were twenty-seven patients in the Paraneoplastic neurologic syndrome groups whose ANA were positive. The mean titer of ANA was 1:640.There were fourteen patients in the twenty-nine Paraneoplasytic Peripheral neuropathy patients groups whose ANA were positive. The mean titer of ANA was 1:320.While,in the control groups,ANA were all negative. Conclusions ESR and CRP can elevated in Paraneoplastic neurologic syndrome patients without specificance. There is no significance of NSE in the diagnosis of Paraneoplastic neurologic syndrome. But for those whose ANA are positive,Paraneoplastic neurologic syndrome should be thought about when there is no evidence of connective tissue disease.Keywords:
Enolase
Erythrocyte sedimentation rate
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The clinical significance of striational antibodies (StrAbs) detected in the course of paraneoplastic antibody testing is unknown.We compared all 203 striational antibody (StrAb)-seropositive patients identified (2004-2005) during evaluation for paraneoplastic antibodies with age- and sex-matched seronegative controls.Thymoma and myasthenia gravis (MG) were significantly more common among cases (P<0.0001). Cancers more rarely detected after StrAb detection were adenocarcinoma in 5 patients and sarcoma in 3 patients. All patients who had a cancer identified after StrAb testing had a titer of ≥ 1:7680 or a coexisting muscle AChR-binding antibody. Autoimmune disorders more commonly observed among cases (with any StrAb value) included: hypothyroidism; rheumatoid arthritis; and pernicious anemia (all P<0.05).StrAbs may serve as a diagnostic clue for an autoimmune diagnosis. There is a low likelihood of oncological significance in patients with StrAb titers <1:7680 without coexisting paraneoplastic Abs.
Clinical Significance
Antibody titer
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Clinical significance of specific antinuclear antibodies (ANA) in Japanese patients with systemic sclerosis was studied. The patients with systemic sclerosis were classified into four groups according to ANA: (1) anticentromere antibody-positive; (2) anti-Scl-70 antibody-positive; (3) anti-nRNP antibody-positive, and (4) others. The mean score (the number of positive signs in six selected specific signs) of the patients with anti-Scl-70 antibody was significantly higher than those of the other three groups. More frequent contracture of phalanges in the patients with anti-Scl-70 antibody less diffuse pigmentation in the patients with anti-nRNP antibody and less pulmonary fibrosis in the patients with anticentromere antibody were revealed. As shown above the detection of specific ANA in systemic sclerosis is clinically important.
Scleroderma (fungus)
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Objective: There is no clear data on autoantibody levels in Kawasaki Disease (KD) especially from the Indian Subcontinent. Aim: To look for the presence of organ nonspecific and organ specific antibodies to strengthen the search for an autoimmune cause of KD. We tested the presence of antinuclear antibody (ANA) and antithyroid microsomal antibody (TMA) in children with KD, 6 months after the acute phase. Anti Neutrophil Cytoplasmic Antibody (pANCA, cANCA), Anti Endothelial Cytoplasm Antibody (AECA) and Anti Smooth Muscle Antibody (SMA) was additionally tested in those with elevated titers of ANA and/or TMA. Materials and Methods: Prospective case-control study of 24 children with KD on follow up and an equal number of age and sex matched controls. Historical data about acute phase of illness was obtained from the medical records. After obtaining institutional ethics committee clearance and informed consent from the parents, blood was tested for ANA and TMA by the indirect immunofluorescence method (IIF), using a kit developed by Euroimmun. Positive samples were additionally tested for pANCA, cANCA, AECA and SMA. Relationship of autoantibody elevation and clinical course in the cases was determined. Results: The age of the study group was 4 3.2 years. Incomplete KD was seen in 12.5% of the cases. Five cases (21%) had cardiac involvement. All but one with mitral and tricuspid regurgitation resolved after the acute phase of the disease. Only her ANA was elevated. Two children (8%) positive for TMA did not show any cardiac abnormalities. Further antibody testing was negative. All three children with elevated autoantibodies were females. (P value = 0.02: statistically significant). Conclusion: Elevated autoantibodies in three (12.5%) children after the acute phase may suggest the role of autoimmunity in the etiopathogenesis of KD, even though our observations were not statistically significant.
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Some patients with rheumatoid arthritis (RA) as well as those with other collagen diseases are positive for antinuclear antibody (ANA). We investigated the frequency of positivity for ANA in 104 patients with RA and evaluated the clinical features and laboratory data in the ANA-positive and -negative groups. The presence of ANA in sera was studied by indirect immunofluorescence using HEp-2 cells as the antigen substrate. Sera with a positive fluorescence at a dilution of 1:20 were considered to be positive for ANA. Of the 104 patients, 39 (37.5%) were positive for ANA. The staining pattern in the positive cases varied, but most were speckled (64.1%) and homogeneous (48.7%). A small number showed a nucleolar (20.5%) or a centromere (10.3%) pattern. None showed a shaggy pattern. The ANA titer was lower in RA patients compared with those with other collagen-related diseases such as systemic lupus erythematosus or progressive systematic sclerosis. None of the patients positive for ANA with either a nucleolar or centromere staining pattern had progressive systemic sclerosis or the CREST syndrome. One patient each had Raynaud's phenomenon and pulmonary fibrosis. There was no correlation between ANA positivity and indicators of joint inflammation. The prevalence of ANA positivity in patients with advanced or prolonged disease was higher than those with early stages or short durations. There was no correlation with drug therapy.
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Scleroderma (fungus)
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Abstract Objective . To clarify the clinical features and prognosis of systemic sclerosis (SSc) based on serum antinuclear antibodies (ANA). Methods . We studied 275 consecutive Japanese patients newly diagnosed as having SSc, who were first evaluated during the period 1971—1990. Eight SSc–related ANA were identified using indirect immunofluorescence, double immunodiffusion, or immunoprecipitation assays. Clinical and prognostic features were retrospectively analyzed in patient groups, categorized by their serum ANA. Results . Cumulative survival rates at 10 years after diagnosis of SSc were 93% in patients with anticentromere antibodies (ACA), 72% in those with anti—U1 RNP, 66% in those with anti—DNA topoisomerase I (anti—topo I), and 30% in those with anti‐RNA polymerases I, II, and III (anti‐RNAP). Major organ involvement linked to cause of death included biliary cirrhosis in patients with ACA, isolated pulmonary arterial hypertension and cerebral hemorrhage in those with anti—U1 RNP, pulmonary interstitial fibrosis in those with anti—topo I, and cardiac and renal involvement in those with anti‐RNAP. Conclusion . Determinations of serum ANA in SSc patients are useful in predicting organ involvement and long‐term outcome.
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Introduction: Antinuclear antibodies (ANA) are autoantibodies directed against different constituents of the cell. Chronic interstitial lung diseases (ILD) are a heterogeneous group of disorders with multiple etiologies. The objective of the study was to estimate the prevalence of ANA during ILD and to determine predictive factors of their presence. Methods: It was a retrospective study including patients with confirmed ILD hospitalized in respiratory department D, between 2011 and 2016 and in whom ANA were detected at the immunology laboratory of Abderrahmane Mamie Hospital. Results: Seventy-three patients were included. The average age was 62 (29 - 90 years). A female predominance was noted (sex ratio H/F=0.4). The main etiologies found were idiopathic interstitial pneumonia (59%), connective tissue disease (23%) and sarcoidosis (5.4%). The main connective tissue diseases found were rheumatoid arthritis (n=6), antisynthetase syndrome (n=2), Sjogren9s syndrome (n=3) and systemic lupus erythematosus (n=3). The prevalence of ANA was 31.5%. The ANA title ranges from 1/80 to 1/1280. The ANA's patterns were homogeneous (n=8), speckled (n=8), nucleolus (n=4) and cytoplasmic (n=3). The typing of ANA identified anti-SSA Ro52 in 7 patients, anti RNP in 2 patients, anti Jo1 in one patient, anti-Ribosome-P in one patient and anti Scl 70 in one patient. Associated factors with ANA were skin manifestations (25% vs 6%, p=0.046) and sedimentation rate ≥ 70 s. Conclusion: During ILD, ANA were detected in the third of patients. Skin manifestations and increased sedimentation rate seem to be predictive factors of their positivity.
Antisynthetase syndrome
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Rheumatoid factor
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Neuroradiology
Neurological examination
Positive correlation
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Abstract Sera from 47 patients with PSS were studied for the presence of antinuclear antibodies. Antinuclear antibodies were present in 60 per cent of the sera in a titer of 1 :16 or greater. In most cases the titer was low, but a titer of 1 :256 or greater was present in sera from 6 patients. The pattern of nuclear fluorescence was most commonly that of fine or large speckles. Most sera contained both IgC and IgM antinuclear antibodies. There was no relation between duration of disease or severity of disease and the presence of antinuclear antibodies. Similarly, no correlation could be demonstrated between clinical findings and the immunoglobulin class, titer, or staining pattern of antinuclear antibody. The prevalence of high tilers of antinuclear antibodies was greater in patients with hypergammaglobulinemia than in those with normal levels of gamma globulin. Rheumatoid factor was present in 33 per cent of sera. Patients with high liters of rheumatoid factor tended to have high tilers of antinuclear antibodies. Antinuclear antibodies were found in low titer in 7 per cent of blood relatives less than 60 years of age. The number of sera with antinuclear antibodies was slightly higher in the non‐aged relatives of patients with systemic lupus erythematosus (11 per cent), while the prevalence in relatives of patients with chronic discoid lupus (2 per cent) was similar to that of a group of healthy student nurses. The results reveal that antinuclear antibodies of a speckled pattern are commonly present in sera from patients with FSS and appear to be unrelated to the duration or severity of the disease. The peripheral pattern of nuclear fluorescence produced by sera from acutely ill patients with systemic lupus erythematosus was not observed with any of the sera of patients with PSS. These obs 3 ervations emphasize the fact that the presence of antinuclear antibodies in general is not diagnostic of any one disease, and indicate that the pattern of nuclear fluorescence produced by the antinuclear antibodies may aid in confirmation of clinical diagnosis.
Rheumatoid factor
Scleroderma (fungus)
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Antinuclear antibodies (ANA) are the main serologic markers of type 1 autoimmune hepatitis (AIH); however 20-30% of patients are negative for ANA. We assessed the clinical features of ANA-negative patients.A retrospective analysis was performed of 176 patients with type 1 AIH (153 females, median age 55 years). A diagnosis of AIH was made based on the revised scoring system proposed by the International Autoimmune Hepatitis Group. ANA titers were measured using a standard indirect immunofluorescence technique.Thirty-eight patients (22%) had low titers of ANA (1:40 or 1:80), and 114 (65%) had high titers (>/= 1:160). Of 24 ANA-negative patients, 15 were positive for smooth muscle antibodies (SMA). Three of nine both ANA- and SMA-negative patients developed ANA during follow-up. The other six were diagnosed based on histological characteristics. Thirteen ANA-negative patients relapsed after the normalization of serum alanine aminotransferase (ALT) levels. ANA-negative patients more frequently showed acute presentation and, at presentation, had lower serum immunoglobulin G levels, higher serum levels of bilirubin and transaminase, and higher frequencies of histological acute hepatitis and zone 3 necrosis than those with high titers. However, the frequency of advanced stage of fibrosis was similar. The response to corticosteroids was not different among the three groups.ANA-negative type 1 AIH shows acute-onset more frequently but may include not only acute autoimmune hepatitis, but also acute exacerbation of inactive chronic disease. Regarding the diagnosis of ANA-negative AIH, the determination of ANA during follow-up and the response to immunosuppressive treatment may be helpful.
Autoimmune Hepatitis
Overlap syndrome
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Background
Proliferating cell nuclear antigen (PCNA) antibodies are a rare type of antinuclear antibodies (ANA), historically considered highly specific for systemic lupus erythematosus (SLE). Recent studies showed they can be present in other autoimmune diseases, viral infections or tumors. Our aim was to clinically describe patients positive for anti-PCNA antibodies.Methods
We retrospectively identified patients with a PCNA pattern by indirect immunofluorescence (IIF), subsequently confirmed by immunoblot, selected among patients tested for ANA, between January 2006 and August 2019. Clinical and analytical data were obtained from medical records.Results
11014 samples (excluding follow-up tests) were tested for ANA, 2642 (23.99%) were positive (titer >1/160), 26 (0.98%) had a PCNA pattern by IIF but only 6 (0.23%) were anti-PCNA positive. Mean patients' age was 51.5±17.93 years-old and the majority were female (83%). Half had SLE (including one Overlap Syndrome). Other autoimmune diseases included Antiphospholipid syndrome [n=2, 33%], Systemic Sclerosis [n=1, 17%] and Behçet Disease [n=1, 17%]. One patient developed metastasis from an occult neoplasia and none had viral disease. Almost all patients had cutaneous manifestations [n=5, 83%]. Half had articular [n=3, 50%] and neurological manifestations [n=3, 50%]. Median ESR and RCP were slightly elevated. None had complement consumption. Mean GFR was normal, with one patient showing low level of proteinuria. Anti-dsDNA, anti-SSA, anti-M2 and anti-cardiolipin were also found in the sera. Two patients were under hydroxychloroquine [n=2, 33%] and one under prednisolone 10 mg [n=1, 17%] (table 1).Conclusions
Our study confirmed that anti-PCNA antibodies can be present in other autoimmune diseases and are not SLE specific. Given its rare prevalence, evidence about the clinical relevance of anti-PCNA antibodies is lacking. Larger studies are needed to further evaluate its role. Skin: Malar rash and/or photosensitivity and/or oral/genital ulcers and/or Raynaud phenomenon and/or telangiectasias and/or microstomy and/or digital ulcers and/or skin sclerosis; Joints: Arthralgias and/or arthritis; Neurological: Focal neurological symptoms and/or dementia and/or cerebrovascular disease; Constitutional: Fatigue and/or weight loss; Renal: serum creatinine elevation and/or proteinuria and/or hematuria; Muscle: Myalgias and/or myositis; Sicca: Dry mouth/eyes; RCP: Reactive C protein; ESR: Erythrocyte sedimentation rate; GFR: Glomerular filtration rate.IIf
Hydroxychloroquine
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