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G Pokorny

Publications and source records attributed to G Pokorny.

At least 19 recordsLinked to original sources
PubMed2005

Clinical associations of autoantibodies to human muscarinic acetylcholine receptor 3(213-228) in primary Sjogren's syndrome.

OBJECTIVES: The authors have previously identified a peptide of the human muscarinic acetylcholine receptor-3 (m3AChR) as a suitable antigen for the immunodetection of antimuscarinic acetylcholine receptor autoantibodies in primary Sjögren's syndrome (pSS). The aim of this study was to assess the clinical correlations and disease specificity of these antibodies. METHODS: Seventy-three pSS, 40 rheumatoid arthritis (RA), 19 systemic lupus erythematosus (SLE), 14 secondary Sjögren's syndrome (sSS) patients, 22 subjects in whom pSS was suspected but in whom the diagnosis not could eventually be established (suspSS) and 40 healthy subjects were investigated. An enzyme-linked immunosorbent assay system developed by the authors using a 16-mer peptide of the m3AChR (m3AChR(213-228)) in a recombinant fusion peptide form was used as the antigen. RESULTS: Anti-m3AChR(213-228) antibody positivity was observed in 66 (90%) of the pSS patients. The antibody levels correlated positively with the number of extraglandular organ manifestations. Both the mean antibody levels and the occurrence of anti-m3AChR(213-228) positivity were significantly higher in pSS than in the comparison groups. The test discriminated the pSS patients from the various comparison groups with specificities of 65, 68, 71 and 50% for RA, SLE, sSS and suspSS, respectively. CONCLUSIONS: The presence of m3AChR(213-228) antibodies is a common feature in pSS. Although it is significantly more common in pSS than in the comparison groups, anti-m3AChR(213-228) positivity is not exclusive to pSS.

Adult↗
PubMed2002

Antineutrophil cytoplasmic antibodies in patients with systemic lupus erythematosus.

We determined the prevalence of antineutrophil cytoplasmic antibodies (ANCAs) in patients with systemic lupus erythematosus (SLE) and evaluated the correlation between ANCA positivity and clinical features. Forty-one patients with SLE and two control groups were examined. One of the control groups consisted of 15 patients with systemic vasculitis, and the other of 12 healthy blood donors. A quantitative enzyme-linked immunosorbent assay technique was used to measure the serum cytoplasmic ANCA (cANCA) and perinuclear ANCA (pANCA) levels. cANCA positivity was found in three patient samples, and pANCA positivity in 10 SLE patients. The occurrence and titres of both ANCA types in SLE patients were similar to those in healthy controls and significantly lower than those in patients with systemic vasculitis. The clinical picture and antibody profile were similar in ANCA-positive and ANCA-negative SLE patients. We conclude that measurement of ANCAs does not provide any additional diagnostic or prognostic data in SLE.

Adult↗
PubMed2001

Incomplete lupus erythematosus: results of a multicentre study under the supervision of the EULAR Standing Committee on International Clinical Studies Including Therapeutic Trials (ESCISIT).

OBJECTIVE: Patients characterized with antinuclear antibodies (ANA) and disease symptoms related to one organ system can be described as having incomplete systemic lupus erythematosus (SLE). The aim of this multicentre study was to describe the outcome of these so-called incomplete SLE patients. Two aspects of the outcome were studied: (i) the disease course, defined by the presence or absence of clinical symptoms; and (ii) the number of patients that eventually developed full SLE. METHODS: Outcome parameters were the ACR criteria, the SLE disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measure (ECLAM) and the requirement for treatment. In 10 European rheumatology centres, patients who had been evaluated in the last 3 months of 1994 and had been diagnosed as having incomplete SLE on clinical grounds for at least 1 yr were included in the study. All 122 patients who were included in the study were evaluated annually during 3 yr of follow-up. RESULTS: Our results are confined to a patient cohort defined by disease duration of at least 1 yr, being under clinical care at the different centres in Europe. These patients showed disease activity that was related mostly to symptoms of the skin and the musculoskeletal system, and leucocytopenia. During the follow-up, low doses of prednisolone were still being prescribed in 43% of the patients. On recruitment to the study, 22 of the 122 incomplete SLE patients already fulfilled the ACR criteria for the diagnosis of SLE. In the 3 yr of follow-up only three patients developed SLE. CONCLUSIONS: A high proportion of patients in our cohort defined on clinical grounds as having incomplete SLE eventually showed disease activity defined by the SLEDAI as well as ECLAM. However, only three cases developed to SLE during the follow-up. This suggests that incomplete SLE forms a subgroup of SLE that has a good prognosis.

Adolescent↗
PubMed2001

Systemic lupus erythematosus. Disease outcome in patients with a disease duration of at least 10 years: second evaluation.

Data related to the disease course of patients with systemic lupus erythematosus (SLE) with special attention to the persistence of disease activity in the long term are scarce. At this moment reliable figures are only known about the survival rate as a measure of outcome. The aim of this multicenter study was to describe the outcome of SLE patients with a disease duration of greater than 10 y. Outcome parameters were two disease activity-scoring systems (SLEDAI and ECLAM), the end organ damage (SLICC/ACR damage index) and treatment. Our results are derived from 187 SLE patients followed at 10 different centres in Europe over a period of 1 y. Serious clinical signs or exacerbations, defined by the occurrence or detoriation of already existing symptoms of renal and cerebral nervous systems were observed in 2-11% of the patients, seizures and psychosis in 3%, proteinuria in 11% and an increase in serum creatinine in 5% of the patients. No change took place in the overall damage index. Yet, the disease course in most patients was characterized by periods of tiredness (42-60%), arthritis (20-25%), skin involvement such as malar rash (32-40%), migraine (15-20%), anaemia (15%) and leucopenia (17-19%). Summarizing these results it is shown that patients, still under care after such a long time of having this disease, do have a disease that is far from extinguished.

Adult↗
PubMed2000

[Diffuse alveolar hemorrhage in systemic lupus erythematosus].

Of the 120 systemic lupus erythematosus (SLE) patients treated by the authors, two have developed diffuse alveolar haemorrhage. The authors' objective is to present this rare, but severe manifestation. Patients 1 and 2 were 66- and 22-year old women, respectively. Both had SLE with multi-organ involvements including diffuse proliferative lupus nephritis. Before the diagnosis of the disease, both patients had experienced pneumonitis that resolved on corticosteroid treatment. Soon after the diagnosis, respiratory failure, haemoptoea and acute anaemia developed, accompanied by a rapid deterioration in the general condition. Chest radiographs revealed bilateral, diffuse, alveolar infiltrates. The pulmonary haemorrhage temporarily ceased in response to corticosteroid treatment, but both patients later died in consequence of active SLE and mixed bacterial and fungal sepsis. Post mortem examination demonstrated fibrosing alveolitis and alveolar bleeding in Patient 1, and an immune complex deposition-induced alveolocapillary inflammation with alveolar haemorrhage in Patient 2. Diffuse alveolar haemorrhage is a life-threatening manifestation of SLE. Its onset may be preceded by episodes of pneumonitis resolving on corticosteroid treatment. An active diagnostic workup, intensive observation and aggressive immunosuppressive treatment are the cornerstones of the management. The early detection and the active treatment of secondary infections are obligatory. The authors consider the most difficult challenge to be the optimum coordination of the above treatment modalities.

Adult↗
PubMed2000

The place of magnetic resonance and ultrasonographic examinations of the parotid gland in the diagnosis and follow-up of primary Sjögren's syndrome.

OBJECTIVE: The aim was to determine the place of magnetic resonance imaging (MRI) and ultrasonographic (US) examination in the diagnosis and follow-up of Sjögren's syndrome (SS). METHODS: Parotid MRI and US examinations were carried out on 44 primary SS patients and 52 controls of similar age. RESULTS: The most important structural changes in SS were different degrees of parenchymal inhomogeneity, which could be detected by both methods, and were found more frequently in the SS patients than in the controls (MRI: 95.4 vs 17. 3%; US: 88.6 vs 7.7%; P<0.001). There was good agreement between the MRI and US findings both in the SS cases (93.2%) and in the controls (86.5%). In one SS patient who developed parotid lymphoma, the US examination showed a hypoechoic 'cobblestones'-like inhomogeneous internal pattern which was coupled with an almost homogeneous MRI pattern. CONCLUSIONS: MRI appears unnecessary as a routine method in the diagnosis of SS; US examination is suitable both for the diagnosis and follow-up of SS. The above combination of the seemingly contradictory US and MRI findings is highly characteristic of lymphoma which has developed in the course of the disease.

Adult↗
PubMed2000

Impaired microvascular response to cholinergic stimuli in primary Sjögren's syndrome.

OBJECTIVE: Signs of a parasympathetic dysfunction have been revealed in primary Sjögren's syndrome (SS). Its role in the pathogenesis and the clinical picture of the disease is not clear. To investigate the responsiveness of SS patients to a cholinergic agonist, a model was used involving examination of the cutaneous microcirculation. The microvascular response to the administration of carbachol was measured, a muscarinic cholinergic agonist. METHODS: Twenty two SS patients and 12 controls were examined. Carbachol and 0.9% saline solution were administered intracutaneously into the forearm skin at two distinct places. Skin blood flow (SBF) in the injected areas was measured continuously before and for 10 minutes after the injections by means of a laser Doppler perfusion monitor. The increase in SBF in response to carbachol (dSBF), reflecting vasodilatation, was calculated by a formula including the baseline and the maximum SBF values after the injections of carbachol and saline solution. RESULTS: The vasodilatation was significantly lower in SS patients than in the controls (mean dSBF: 2.1 (range: 1.0-4.5) versus 3.3 (range: 1.7-7.6), p=0.02). With non-responder patients defined as those in whom a smaller response was observed than in any of the controls, 11 of the 22 SS patients proved to be non-responders to carbachol. Comparisons of demographic, clinical and laboratory characteristics and HLA class II genotypes between responder and non-responder SS patients did not show any significant differences. CONCLUSIONS: A diminished or absent response to carbachol indicates a cholinergic dysfunction in SS patients. A disturbance in the neurotransmission at a receptorial or postreceptorial level is hypothesised. Unresponsiveness to cholinergic stimuli may contribute to exocrine insufficiency.

Adult↗
PubMed2000

Relationship between the occurrence of anti-SSA, anti-SSB autoantibodies and HLA class II alleles from the aspect of in vitro inhibitory effect of glucocorticosteroid on the antibody-dependent cellular cytotoxicity in patients with primary Sjögren's syndrome.

Though at present there is no evidence-based algorithm for the treatment of primary Sjögren's syndrome, it is generally accepted that glucocorticosteroid (GS) therapy must be introduced in cases with severe systemic manifestations. As the side-effects of the GSs are well known, it would be useful to know in advance how the patients will respond to this type of treatment. For this reason we measured the in vitro steroid sensitivity of 29 SS patients using inhibition of antibody dependent cellular cytotoxicity (ADCC) test by methylprednisolone compared to that of 28 controls. SS patients proved to be significantly less sensitive to GSs than controls (inhibition of ADCC reaction: 42.4 vs 53.1%; p < 0.01). This was especially true in SS patients with anti-SSA and/or SSB autoantibody positivity and with HLA-DR2 and/or -DR3 alleles. Comparing the results of the in vitro GS sensitivity and the clinical effectiveness of the previously applied corticosteroid therapy it seems that steroid inhibition of ADCC reaction has a predictive value in determination of in vivo sensitivity to GSs. However, in patients with decreased in vitro GS sensitivity a more expressed in vivo steroid sensitivity cannot be excluded.

Adult↗
PubMed1999

Systemic lupus erythematosus: clinical features in patients with a disease duration of over 10 years, first evaluation.

OBJECTIVE: Most information available about the disease course of patients with systemic lupus erythematosus (SLE) is restricted to the first 5 yr after disease onset. Data about the disease course 10 yr after disease onset are rare. The aim of this multicentre study was to describe the outcome of SLE patients with a disease duration of >10 yr. METHODS: Outcome parameters were the SLE Disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measure (ECLAM), the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR), a global damage index (DI) and required treatment. In 10 different European rheumatology centres, all SLE patients who were evaluated in the last 3 months of 1994, and who had been diagnosed with SLE at least 10 yr ago, were included in the study. RESULTS: It should be stressed that our results are confined to a patient cohort, defined by a disease duration of at least 10 yr, and who are still under clinical care at the different centres in Europe. These SLE patients still showed some disease activity, related to symptoms of the skin and musculoskeletal systems, next to the presence of renal involvement. A total of 72% of the patients needed treatment with prednisolone (</=7.5 mg). The cumulative damage was overall related to clinical features of the central nervous system (14%) and renal involvement (14%), next to deforming arthritis (14%), osteoporosis (15%) and hypertension (40%). The prevalences of obesity, Cushing appearance and diabetes are highly suggestive that the ongoing treatment and that in the past might have had an impact on the total sum of end-organ damage. CONCLUSIONS: After 10 yr, a high proportion of patients in our cohort continued to show evidence of active disease, defined by the SLEDAI as well as ECLAM. The DI was related to the involvement of the central nervous system, renal involvement and the presence of hypertension.

Adult↗
PubMed1999

Antineutrophil cytoplasmic antibodies and other immunologic abnormalities in patients with habitual abortion.

PROBLEM: The immunologic mechanisms of pregnancy loss in habitual aborters with antiphospholipid and antinuclear antibodies have not been fully clarified. The possible association of antineutrophil cytoplasmic antibodies (ANCAs) with recurrent miscarriage was examined. METHOD OF STUDY: In a prospective, controlled trial of 59 women with recurrent abortion, the prevalence of pANCA (antimyeloperoxidase), cANCA (antiproteinase-3), and immunoserologic abnormalities of systemic lupus erythematosus (SLE) anti double-stranded DNA, anti-SSA, anti-SSB, anti-U1RNP, anti-Sm, anticardiolipin and antinuclear antibodies, LE-cell, lupus anticoagulant, and complement-3 were investigated. RESULTS: pANCA occurred in 2, and cANCA in 6 of 59 case patients, but neither was observed in the controls (P = 0.09 for cANCA). cANCA levels were significantly higher in patients than in controls (P = 0.028). Six recurrent aborters were identified as having a group of immunoserologic abnormalities characteristic of SLE. CONCLUSIONS: Immunologic mechanisms detectable in SLE may operate in a subgroup of habitual aborters with suspected immunologic cause. ANCAs occur more frequently in patients with recurrent miscarriage than in controls.

Abortion, Habitual↗
PubMed1998

Polyneuropathy with endoneurial immune complex deposition as the first manifestation of systemic lupus erythematosus.

A 72-year-old male presented with progressive sensorimotor polyneuropathy. Later weight loss, proteinuria and deteriorating renal function were noted. The electrophysiological examinations revealed extensive, symmetrical demyelinating and axonal polyneuropathy. Frozen sections obtained from sural nerve biopsy sample showed the presence of immune complexes and complements in the walls of the epi- and endoneurial blood vessels, and perineurium suggestive of systemic lupus erythematous (SLE). IgG and Clq deposits were also present along the basement membranes of Schwann cells. The electron microscopy confirmed the presence of immune complex deposition. Diagnosis of SLE was proven by positive serology (anti-nuclear antibodies, anti-Sm, anti-RNP, anti-double-stranded DNA) and renal biopsy showing membranous lupus nephritis with extensive immune complex deposition in the tubular basement membranes. Despite combined immunosuppressive treatment for 10 months, the patient died of complications of generalized immune complex vasculitis. The manifestation of SLE in elderly patients, especially in males, is very rare. Moreover, the polyneuropathy is an unusual initial symptom of SLE. Immune complex deposition in Schwann cell basement membrane probably plays an important role in the pathomechanism of sensorimotor polyneuropathy in SLE.

Aged↗
PubMed1996

Parotid gland ultrasonography as a diagnostic tool in primary Sjögren's syndrome.

The diagnostic value of parotid gland ultrasonography (Acuson 128, 7 MHz transducer) was studied in 62 patients with primary Sjögren's syndrome (SS) and in 69 controls of similar age and sex distribution. Different degrees (mild, evident or gross) of parenchymal inhomogeneity (PIH) were the most important sonographic changes in SS; they occurred in 83.9% of the patients. The sonographic results (the presence or absence of PIH) were in accordance with the parotid sialographic and scintigraphic findings and the histology of the minor salivary glands in 87.3, 84.7 and 84.3% of the cases, respectively. Of the degrees of PIH, only evident and gross PIH are thought to be of true diagnostic value for SS. On the basis of the good agreement between the sonographic and sialographic results, consideration of the introduction of parotid sonography as an alternative to sialography is suggested in SS if the latter method cannot be performed.

Adult↗
PubMed1996

Cardiac manifestations in primary Sjögren's syndrome.

OBJECTIVE: To determine cardiac manifestations in primary Sjögren's syndrome (SS). METHODS: Echocardiographic examination was undertaken in 64 patients (62 women, two men) with primary SS (54 definite (DSS) and 10 probable (PSS)) who had systemic symptoms. Twenty one healthy women volunteers of similar age acted as controls. RESULTS: Acute exudative pericarditis occurred in only one patient. An echogenic pericardium was demonstrated in 21 patients (19 DSS, two PSS) (33%) who had a previous symptom free pericarditis, but in none of the controls. Pulmonary pressure was significantly greater in the patients than in the controls (31 (SD 8) mm Hg compared with 24 (7) mm Hg), but there was no significant difference between the DSS and PSS groups. Left ventricular (LV) systolic function was similar in patients and controls. Twenty two patients (20 DSS, two PSS) and one control subject were excluded from LV diastolic function evaluation because of conditions likely to influence the parameters. Of the remaining 42 patients with SS (34 DSS, eight PSS), 21 (17 DSS, four PSS) had impaired diastolic function, confirmed by several diastolic parameters. LV diastolic dysfunction and echogenic pericardium occurred independently of each other, and there was no correlation between the occurrence of these silent cardiac abnormalities and the clinical and laboratory findings. CONCLUSIONS: Obvious cardiac involvement is rare in primary SS, but clinically silent manifestations (symptom free pericarditis and LV diastolic dysfunction) are common. The clinical and prognostic significance of these changes cannot yet be defined.

Acute Disease↗
PubMed1991

[Secondary membranous glomerulonephritis].

Following 689 percutaneous renal biopsies, membranous glomerulonephritis was proved in 68 patients. In 16 (23.5%) an underlying primary disease was verified, and thus the glomerulonephritis the secondary form. The primary disease was SLE in 5 cases, diabetes mellitus in 5 cases, rheumatoid arthritis in 3 cases, chronic active hepatitis in 2 cases, an ulcerative colitis and eosinophilic angiolymphoid hyperplasia in 1 patient. As initial sign, nephrotic syndrome emerged in 87.5% of the 16 cases. Microscopic haematuria was observed in half of the patients, as was hypertension, while acute renal failure presented in only 1 case. Histologically, in 13 cases the predominance of early glomerular alterations was characteristic, while in 9 cases the picture proved to be equivocal and accompanied by some degree of interstitial alterations. During combined treatment, remission was achieved in 75%. Two patients with SLE died, but not as a consequence of renal failure. Transient side-effects of the treatment were registered in 5 cases. The principal pathogenetic and clinical differences between the individual secondary nephritis forms, and the difficulty of their differentiation from the idiopathic cases, even on repeated examination, are emphasized. In 3 patients the possibility of secondary renal processes was suggested by the histological picture, and this was proved by the detailed clinical findings.

Adult↗
PubMed1991

Primary Sjögren's syndrome from the viewpoint of an internal physician.

The characteristics of primary Sjögren's syndrome are described on the basis of the follow-up of 65 patients with extraglandular symptoms at the onset and during the disease. The mean age of the patients at onset was 41.8 years and at the time of definite diagnosis was 45.8 years. Articular (32 cases), lacrimal (30 cases) and salivary (30 cases) manifestations were the most frequent initial symptoms. In only 22 of the 65 patients could Sjögren's syndrome be diagnosed at the onset. In most cases, the articular symptoms observed observed in 56 patients during the course corresponded to true polyarthritis, as verified by joint scintigraphy. Most frequently the wrists and ankles were affected. Chronic atrophic gastritis was found in 35 patients. In the young patients (13 cases), both the antrum and the corpus were affected more frequently than in the controls. In middle-aged patients (21 cases), atrophy of the antrum, and in the elderly (10 cases) atrophy of the corpus was more frequent than in the controls. All three types of chronic atrophic gastritis occurred in the disease. The decreased gastric acid secretion was characteristic of types A and AB gastritis, but the hypergastrinaemia only of type A. It was verified that chronic duodenitis and jejunitis occur in the disease. The pancreatic lesions were mild. Renal involvement was detected in 15 patients, vascular symptoms in 22 and lower-airway changes in 21. The variety of the different symptoms proved that primary Sjögren's syndrome can involve many organs.

Acidosis, Renal Tubular↗
PubMed1991

Types of atrophic gastritis in patients with primary Sjögren's syndrome.

Histological examination of the gastric mucosa was performed in 44 patients with primary Sjögren's syndrome with extraglandular symptoms (mean age 51.9, range 22-76). Biopsy specimens were taken from each of three separate regions: the antrum, the corpus, and the transitional zone between the antrum and the corpus. The incidence of chronic atrophic gastritis was considerably higher in patients with Sjögren's syndrome than in the controls. In the young patients with Sjögren's syndrome atrophic lesions were more common both in the antrum and in the corpus than in the control group. In middle aged patients, however, only the antrum, and in the elderly only the corpus, was much more commonly affected than in the controls. All three types of chronic atrophic gastritis occurred in patients with Sjögren's syndrome. Decreased gastric acid secretion was associated mainly with atrophic gastritis of types A and AB, whereas hypergastrinaemia occurred almost exclusively in gastritis of type A.

Adult↗