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Biomedical subjects

T Chorzelski

Publications and source records attributed to T Chorzelski.

At least 19 recordsLinked to original sources

The clinical transition between pemphigus foliaceus and pemphigus vulgaris correlates well with the changes in autoantibody profile assessed by an enzyme-linked immunosorbent assay.

BACKGROUND: There are a number of reports of pemphigus with clinical shifting between pemphigus foliaceus (PF) and pemphigus vulgaris (PV). On the other hand, a novel enzyme-linked immunosorbent assay (ELISA) against recombinant baculoproteins of desmoglein 1 (Dsg1) (PF antigen) and Dsg3 (PV antigen) has been established and found to be extremely sensitive and specific. OBJECTIVES: To characterize the change in the antibody profiles in a series of pemphigus cases with mixed features of PF and PV by various methods, including the novel ELISA. Patients/methods Sera were obtained from eight cases undergoing a shift between PF and PV and three cases of coexistent PF and PV. The autoantigens were analysed by ELISA, as well as by immunofluorescence using normal human skin sections and immunoblotting using normal human epidermal extracts. RESULTS: The results of the ELISA, immunofluorescence and immunoblotting studies showed that the transition between PF and PV correlates well with the changes of autoantibodies against either Dsg1 or Dsg3. CONCLUSIONS: The clinical phenotype at each stage is defined by the anti-Dsg antibody profile in the serum of these pemphigus patients showing mixed features of PF and PV. In addition, ELISA using recombinant baculoproteins was particularly useful in distinguishing PF and PV.

Adult↗

Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.

BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. METHODS: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. Immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. Myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.

Adult↗

[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↗

[The value of IgA endomysium antibodies in diagnosis of coeliac disease among short children].

In recent years a shift in incidence of coeliac disease from the classical to late-onset form has been observed. The main, and often only, symptom of late onset coeliac disease is short stature. The presence of antiendomysial antibodies was found in 14 of 115 children with statural height below the third percentile from randomly chosen kindergartens and schools in Bydgoszcz. Severe atrophy of the intestinal villi in a biopsy specimen confirmed the suspicion of coeliac disease in these children. IgA-EmA are markers of coeliac disease in children with short stature and should be used as a screening test in looking for the causes of short stature.

Adolescent↗

Rare vascular changes in the brain in a case of subacute cutaneous lupus erythematosus.

Histopathological changes in leptomeningeal and cerebral blood vessels in a case of subacute cutaneous lupus erythematosus (SCLE) with the involvement of the central nervous system are presented. A 46-year-old woman died because of cerebral stroke after a 19-year duration of the disease. The general autopsy showed changes in the kidneys, myocardium, spleen and pancreas, typical of systemic lupus erythematosus. The brain autopsy revealed large necrosis in the supply territory of the middle and posterior cerebral arteries in the left hemisphere. In addition, small focal necroses, partially hemorrhagic, were found in both cerebellar hemispheres. Small cortical necrosis was also visible in the right insular area. A diffuse damage of the blood vascular system in the form of fibrinoid necrosis of small sized cerebral blood vessels with inflammatory infiltrates of the vessel wall (necrotizing leukocytoclastic vasculitis) predominated in the microscopic examination of the brain. Vascular changes of vasculopathy type in the form of hyalinization of the vascular wall and fibrinoid necrosis with concomitant numerous, small necroses were observed. In the lumen of left internal carotid artery infiltrated by inflammatory cells, an organized thrombus was found. Giant cells were observed within vascular infiltration.

Arterioles↗

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↗

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↗

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↗

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↗

[Significance of anti-neutrophil cytoplasmic autoantibodies in the diagnosis of Wegener's granulomatosis].

A case of Wegener's granulomatosis (WG) with involvement of the upper and lower respiratory tract and kidneys is presented. Despite specific symptoms the diagnosis was changed several times. Final diagnosis was made with immunofluorescence test for antineutrophil-cytoplasm antibodies (IF-ANCA). In Poland, this is the first report emphasizing the role of ANCA in diagnosis of WG. The significance of this test in diagnosing of WG is presented.

Antibodies, Antineutrophil Cytoplasmic↗

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↗

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↗

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↗

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↗

[Diagnostic value of the lupus band test in patients treated by chronic hemodialysis].

The diagnosis of systemic lupus erythematosus in cases with advanced renal failure is a serious clinical problem. The purpose of the study was an analysis of the incidence of various non-renal criteria (according to ARA) for the diagnosis of SLE in patients with chronic renal failure of various aetiology and find out whether the lupus band test in these patients may serve as an additional criterion for the diagnosis of SLE. The studied group comprised 39 patients with chronic renal failure (28 men and 13 women) aged 17-58 years. In this group 29 cases were treated with dialyses and 10 conservatively. The most frequent clinical sign (apart from renal changes) accepted as diagnostic criteria for the SLE was polyserositis and leucopenia and thrombocytopenia. In no case antinuclear antibodies, antibodies against DNA and against soluble nuclear antigens were found. Positive LBT was obtained in 72.4% of cases with chronic renal failure, particularly frequently in the dialysed patients. A positive result of the LBT cannot be of decisive importance in the diagnosis of SLE but may suggest a need of more detailed investigations (determination of Ro antibodies) for confirmation of the diagnosis.

Adolescent↗