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M Jarzabek-Chorzelska

Publications and source records attributed to M Jarzabek-Chorzelska.

At least 19 recordsLinked to original sources
PubMed2003

Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3.

We describe a case with clinical and histologic features of pemphigus herpetiformis associated with IgG and IgA anti-keratinocyte cell surface antibodies to desmoglein 1 (Dsg1) and exclusively IgG antibodies to desmocollin 3 (Dsc3). The clinical presentation was somewhat similar to IgA pemphigus; the main difference was the prevailing association with IgG antibodies to Dsg1. The presence of IgA anti-Dsg1 antibodies was confirmed by IgA enzyme-linked immunosorbent assay and IgG anti-Dsc3 antibodies were detected by a novel enzyme-linked immunosorbent assay with the use of baculovirus expressing recombinant Dscs for IgG and IgA. The reactivity of IgG antibodies with Dsc3 was confirmed by COS-7 cell cDNA transfection method using cDNA of human Dsc1 to Dsc3. We discuss the differentiation of pemphigus herpetiformis, associated with both IgG and IgA antibodies, from IgA pemphigus, particularly in regard to the autoimmune reaction with Dsgs and Dscs.

Adult↗
PubMed2002

Celiac disease and immunoglobulin a deficiency: how effective are the serological methods of diagnosis?

Immunoglobulin A (IgA) deficiency is 10 to 15 times more common in patients with celiac disease (CD) than in healthy subjects. Serological tests have become the preferred methods of diagnosing CD in both symptomatic and asymptomatic patients. However, commercially available serological methods are limited in that they detect only the IgA isotype of antibodies (with the exception of IgG gliadin assays); hence, IgA-deficient patients with CD may yield false-negative serology. Fifteen pediatric patients with CD and 10 IgA-deficient pediatric patients without CD were examined for IgA and IgG antibodies to endomysium, gliadin, and tissue transglutaminase. Twenty-five specimens from patients with IgA deficiency were examined. Fifteen were from patients with CD, and 10 were patients without CD. All 15 IgA-deficient patients with CD were positive for endomysium antibodies of the IgG isotype and for IgG gliadin antibodies. All but one of the IgA-deficient patients with CD were also positive for IgG tissue transglutaminase antibodies. None of the IgA-deficient patients without CD were positive for any of the antibody markers. All the specimens examined were also negative for IgA-specific antibodies to endomysium, gliadin, and tissue transglutaminase. IgG-specific antibody tests for endomysium, gliadin, and tissue transglutaminase are useful for the identification of IgA-deficient patients with CD. IgG antibody tests along with tests routinely being used in clinical laboratories can reliably detect all active patients with CD. In addition, the levels of these CD-specific IgG antibodies could be used to monitor patient dietary compliance.

Adolescent↗
PubMed2001

Tissue transglutaminase and endomysial antibodies-diagnostic markers of gluten-sensitive enteropathy in dermatitis herpetiformis.

The association of Durhing's disease, commonly referred to as dermatitis herpetiformis (DH), with gluten-sensitive enteropathy (GSE) is supported by the presence of villous atrophy and endomysial antibodies (EMA). EMA are found to be a marker of GSE both in celiac disease (CD) and in DH. Since tissue transglutaminase (tTG) is believed to be the major autoantigen in GSE, the aim of our study was to determine the specificity and sensitivity of anti-tTG antibody ELISA compared to the EMA indirect immunofluorescence test. We studied 44 cases of DH, confirmed by the presence of IgA immune deposits in the dermal papillae, and 58 cases of CD conforming to the International Criteria of Diagnosing CD. The control group comprised 161 sera from patients with vesiculobullous disorders other than DH and 106 sera from normal healthy blood donors. Anti-tTG antibodies were detected in 36 of 44 DH (79%) and in 32 of 58 CD (55%) patients. EMA were positive in 33 of 44 DH (74%) and in 36 of 58 CD (62%) patients. Both the EMA and the antibodies to tTG were present in the majority of patients with DH and CD when they were on a normal gluten-containing diet and were absent when on a gluten-free diet for an extended period of time. There were, however, small discrepancies in positivity and negativity in tTG antibody-positive and EMA-negative patients and vice versa. There seems to be a correlation between the EMA titers and the anti-tTG antibody levels. This study confirms the high specificity and sensitivity of anti-tTG antibody ELISA for GSE and its strong correlation with EMA both in CD and in DH. The results of anti-tTG antibody and EMA assays were comparable; however, in DH, tTG was somewhat more sensitive than the EMA test. For screening of DH, it is advisable to perform both EMA and anti-tTG antibody tests.

Autoantibodies↗
PubMed1999

[Usefulness of analyzing ANCA changes for diagnosis of Wegener's granulomatosis].

UNLABELLED: ANCA were described in 1982 as sensitive and specific markers for active Wegener's granulomatosis (WG). We analysed the results of ANCA test performed in 298 patients hospitalized in Institute of Tuberculosis and Lung Diseases in the period 1990-1998 because of different symptoms and syndromes of respiratory system which could be symptoms of WG. Presence of c-ANCA, p-ANCA and other not well defined types of ANCA in the titer greater than 1:40 in the serum was regarded as positive result of test. We found it in 60 patients. In 47 WG pts ANCA were present in 40 of 42 pts with active disease and 1 of 5 in remission. Further 19 positive results were found in a group of 251 patients with other diseases: 1 of 4 pts with Churg-Strauss syndrome, 1 of 2 with pulmonary renal syndrome, 5 of 28 with connective tissue diseases, 1 of 21 with tuberculosis, 1 of 23 with sarcoidosis, 1 of 6 with histiocytosis, 3 of 11 with hypersensitivity pneumonitis, 1 (lymphoma) of 34 with neoplasms, 1 of 20 with pulmonary fibrosis, 1 of 8 with cardiac failure, 1 of 5 with pleural fluid, 1 of 10 pneumoconiosis and toxic reactions (after furagin), 1 of 6 with BOOP. Sensitivity of ANCA test in our material is 87%, specificity = 95%, and positive prevalence accuracy is 68%. It means that 32% of the patients with positive results could be inappropriate treated as WG. CONCLUSION: ANCA test could be not used as a screening test. Results of ANCA test alone cannot be used as basis for treatment. ANCA test is a helpful tool in diagnosing of WG.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1998

Is chronic ulcerative stomatitis an entity? Clinical and immunological findings in 18 cases.

Chronic ulcerative stomatitis (CUS), a rare disease of oral mucosa found to be associated with unusual antinuclear antibodies reactive exclusively with squamous epithelia (squamous epithelium-specific antinuclear antibodies - SES-ANA), has been reported to occur almost exclusively in older females and to respond dramatically to antimalarials. We report on a large series of 18 cases positive for SES-ANA, 15 of them with active CUS, followed-up for several years. We found that the disease may occur, although rarely, in men and younger females. The antibodies persist after clearance of mucosal lesions, are fixed in vivo and also in uninvolved skin and other mucosa, and may occur in patients without CUS. Thus their pathogenic potential remains to be established. We also describe some unusual cases of CUS, one associated with cicatricial conjunctivitis, another associated with gluten-sensitive enteropathy and bizarre skin lesions, and 3 cases associated with lichen planus (LP) or LP-like lesions. The antimalarials, initially highly effective, even for several months or years, not infrequently proved to be insufficient and the relapses responded usually only to combined therapy with chloroquine and small doses of corticosteroids. SES-ANA antibodies are quite specific and present an immunologic marker of this unusual autoimmune disease.

Adult↗
PubMed1997

Clinical, serologic, and immunogenetic features in Polish patients with idiopathic inflammatory myopathies.

OBJECTIVE: To determine the clinical, serologic, and immunogenetic correlations in patients with idiopathic inflammatory myopathies (IIM), and to evaluate the useful grouping of some diseases for practical clinical purposes. METHODS: Patients with IIM were categorized according to clinical presentation as compared with autoantibody specificity. Serum samples from 84 patients were screened for myositis-specific autoantibodies (MSAs) by indirect immunofluorescence and double immunodiffusion. All sera were also studied by protein A-assisted immunoprecipitation. Genomic DNA was isolated from peripheral blood mononuclear cells, and HLA-DQA1 and DRB1 alleles were determined. The patients were seen and followed up for many years in the same center. RESULTS: MSAs were present in 19% of patients. The most common MSAs were antisynthetases in 13% of patients (Jo-1 10.7%, PL-12 1.2%, and EJ 1.2%), associated with the antisynthetase syndrome. Anti-SRP was found in 1.2% of patients, associated with polymyositis, and anti-Mi-2 in 4.9%, found exclusively in patients with dermatomyositis. The most frequent MSA was PM-Scl in 23.8% of patients, associated with scleromyositis, and Ku was present in 9.6% of patients with overlap syndromes. The alleles that were found at a significantly increased frequency were HLA-DRB1*0301 (59.4%) and DQA1*0501 (71.6%), which are in linkage disequilibrium. DQA1*0501 was present in 85.7% of patients with antisynthetases, and in 100% of patients with PM-Scl and Ku. CONCLUSION: The HLA-DRB1*0301; DQA1*0501 haplotype was found to be significantly increased in this population overall and in those myositis patients with antisynthetase, anti-PM-Scl, and anti-Ku antibodies. The results of this study confirm that IIM are heterogeneous syndromes, but can be divided into more useful groups on the basis of clinical, serologic, and immunogenetic features.

Adult↗
PubMed1996

[Immunoglobulin skin and renal deposits in patients with IgA nephropathy and Schonlein-Henoch purpura].

Skin biopsy was performed in 17 of 27 children with IgA nephropathy (IgAN) and 45 of 62 children with Schönlein-Henoch purpura (SHP). Renal biopsy was done in all patients with IgAN and in 51 of 62 with SHP. There was no correlation between presentation of immunological deposits in skin and renal biopsy. Deposits of IgA with or without other deposits or other deposits without IgA were observed in different pathomorphological states. Correlation between IgA serum concentration and presentation of deposits in skin was not observed. Attention was drawn to the high proportion of negative results in skin biopsy.

Adolescent↗
PubMed1992

Is vascular angiopathy following intracranial aneurysm rupture immunologically mediated?

Immunofluorescence studies showed the presence of IgM and/or C3 in the endothelium of intracranial aneurysms in 5 out of 6 patients with subarachnoid haemorrhage (SAH). In none of them were the immune deposits found in the gyrus rectus. Cortical tissue of 4 epileptic patients which served as a control give negative results. Serum studies on femoral artery wall used as an antigenic substrate did not reveal circulating antibodies of the IgM or IgG class. Our studies strongly suggest that the IgM and/or C3 immune deposits located in the endothelium of intracranial arteries may play a role in post SAH neurological complications.

Adult↗
PubMed1992

Morphea with high titer of fibronectin antibodies.

A 20-year-old man with a 9-year history of unusual subcutaneous morphea with nodular lesions in a circular arrangement and wooden-hard symmetrical induration of the arms, had high titers of fibronectin antibodies. A possible role of fibronectin and antifibronectin antibodies in the pathogenesis of scleroderma is discussed.

Adult↗
PubMed1991

Childhood scleromyositis: an overlap syndrome associated with PM-Scl antibody.

Fourteen patients with childhood scleromyositis followed from 1 to more than 10 years experienced concomitant sclerodermoid and dermatomyositis features, variably expressed at one time or another during the course of the disease. The most characteristic features were myalgia-myositis, arthralgia-arthritis, puffy, atrophic, sclerotic fingers, and Raynaud's phenomenon. This overlap syndrome was the most frequent sclerodermoid condition in children, differing from both systemic scleroderma and dermatomyositis. The course of the disease was protracted and rather benign, and PM-Scl antibody was an important diagnostic and prognostic marker. We present criteria for diagnosis of scleromyositis and its differentiation from systemic scleroderma, dermatomyositis, and Sharp overlap syndrome.

Autoantibodies↗
PubMed1991

Immunologic markers of systemic scleroderma in children.

This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp-2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl-70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl-70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl-70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the significance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.

Adolescent↗
PubMed1991

Immunological markers of the subsets of systemic scleroderma and its overlap.

The studies performed repeatedly in 298 cases of systemic scleroderma (SSc) disclosed high prevalence of antinuclear antibody (in about 95% of the cases) with the use of HEp-2 cells as the substrate for indirect immunofluorescence method. Anticentromere antibody (ACA) was found in 25% of acrosclerosis cases, but in 50% of patients with cutaneous involvement limited to the digits, i.e. almost without cutaneous indurations. The visceral involvement was comparable in cases positive and negative for ACA, and a follow-up of these patients showed that the course, although protracted, was not always mild as previously believed. Scl 70 antibody proved to be a most characteristic marker of SSc, appearing in about 87% of diffuse variety and in above 50% of acrosclerosis cases. The most important finding was the detection of the coexistence of ACA and Scl 70 antibody in 10 of 180 cases studied by three methods: indirect immunofluorescence, double immunodiffusion, (for Scl 70) and immunoblotting with recombinant kinetochore antigen for ACA and recombinant Topoisomerase I antigen for Scl 70 antibody. These antibodies, believed to be mutually exclusive, are most often missed due to masking effect on HEp-2 cells by the stronger antibody, usually Scl 70. Its detection proved to be possible by evaluation of different serum dilutions and several cell mitoses, and confirmed by immunoblotting with recombinant antigens. Of practical significance are the clinical correlations between Pm-Scl antibodies, showing homogeneous nucleolar staining on HEp-2 cells, and overlap or atypical SSc cases with concomitant muscle involvement. This antibody was found to have a surprisingly high association with atypical SSc or scleromyositis of children.

Antibodies, Antinuclear↗
PubMed1990

Basement membrane zone as a target for human neutrophil elastase in psoriasis.

Human neutrophil elastase was found, by indirect immunofluorescence using rabbit anti-elastase anti-serum, to be bound to basement membrane of psoriatic plaques in vivo. The enzyme was also identified inside the migrating neutrophils in the reticular dermis and dermal papillae, as well as outside the cells in micro-abscesses in psoriatic skin. In vitro incubation of normal skin with human neutrophil elastase resulted in the destruction of hemidesmosomes and separation of the epidermis from the dermis above localizations of bullous pemphigoid antigen. These findings are direct evidence that human neutrophil elastase could play a role in psoriasis in in vivo destruction of the epidermal-dermal junction.

Basement Membrane↗
PubMed1990

Antikinetochore and antitopoisomerase I antibodies in systemic scleroderma: comparative study using immunoblotted recombinant antigens, immunofluorescence, and double immunodiffusion.

In 135 patients with systemic scleroderma, we compared three different methods to determine antinuclear autoantibody (ANA) specificity: indirect immunofluorescence, double immunodiffusion, and, employing recombinant antigens, immunoblotting using both marker autoantigens of this disease. A characteristic Scl-70 antibody pattern was found on HEp-2 cells in 83.8% of the patients, double immunodiffusion was positive for the Scl-70 antibodies in 81.9%, and immunoblot with the recombinant topoisomerase I (Topo I) was positive in 71% of the patients. For the centromere autoantibodies we found a high concordance between the anticentromere antibody (ACA) pattern on HEp-2 cells (27 patients positive) and the detection of recombinant kinetochore in immunoblotting (26 patients positive). The three testing techniques gave comparable results, except that the Topo I recombinant antigen used in immunoblotting reacted strongly with fewer than expected of the known Scl-70-positive sera. However, a method using recombinant antigens expressing all epitopes (rather than one of the epitopes of Topo I) will undoubtedly become the method of choice for detecting antibodies in systemic scleroderma. Using the immunoblotting technique with the recombinant antigens we detected in four patients antibodies against both Topo I and kinetochore. More severe symptoms of systemic scleroderma were found in patients who had both antibodies. The combined presence of both marker autoantibodies is therefore not as rare as previously reported and may predict severe disease.

Adolescent↗
PubMed1990

Autoantibodies to nucleolar antigens in systemic scleroderma: clinical correlations.

Indirect immunofluorescence(IIF) and double immunodiffusion (DID) were performed on the sera of 64 patients who had a nucleolar immunofluorescence pattern on HEp-2 cells. Forty-nine of the sera were from 296 patients with systemic scleroderma (SSc) and 15 sera were from 214 patients with systemic lupus erythematosus (SLE). A homogeneous nucleolar staining pattern was found in 45 of the 64 sera (70.3%), a clumpy fluorescence associated with fibrillarin antibody in 14 (21.8%) and a speckled pattern was found in five of the sera (7.8%). There was a clear correlation between the sera which showed a homogeneous nucleolar staining pattern with symptoms of the polymyositis/scleroderma overlap syndrome that differed from SSc with concomitant myositis. The clumpy pattern was mainly associated with diffuse scleroderma and the speckled pattern with limited scleroderma (previously called acrosclerosis).

Adult↗
PubMed1990

Are ACA and Scl 70 antibodies mutually exclusive?

Serological studies using indirect immunofluorescence, double immunodiffusion and immunoblot with recombinant Topo I and kinetochore antigens were performed in 180 patients with systemic scleroderma. In the sera of 10 patients, we found coexisting ACA and Scl 70 antibodies, and this was confirmed by all three methods. The relationship between the coexistence of these antibodies and the clinical features of the disease is not clear, although in all these cases there was pronounced vascular involvement.

Adult↗