Antiphospholipid syndrome accompanied by a silent splenic infarct in a patient with juvenile SLE.
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Antibodies, Anticardiolipin↗
Biomedical subjects
Katalin Dankó
Publications and source records attributed to Katalin Dankó.
Association of systemic and thyroid autoimmune diseases.
OBJECTIVE: There are few large cohort studies available on the association of systemic and thyroid autoimmune diseases. In this study, we wished to determine the association of Hashimoto's thyroiditis (HT) and Graves' disease (GD) with systemic autoimmune diseases. METHODS: One thousand five hundred and seventeen patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), Sjögren's syndrome (SS) and polymyositis/dermatomyositis (PM/DM) were included in the study. The HT and GD were diagnosed based on thorough clinical evaluation, imaging and fine-needle aspiration cytology (FNAC). The frequency of HT and GD in these diseases was assessed. In addition, 426 patients with HT or GD were assessed and the incidence of SLE, RA, SSc, MCTD, SS and PM/DM among these patients was determined. Prevalence ratios indicating the prevalences of GD or HT among our autoimmune patients in comparison to prevalences of GD or HT in the general population were calculated. RESULTS: Altogether 8.2% of systemic autoimmune patients had either HT or GD. MCTD and SS most frequently overlapped with autoimmune thyroid diseases (24 and 10%, respectively). HT was more common among MCTD, SS and RA patients (21, 7 and 6%, respectively) than GD (2.5, 3 and 1.6%, respectively). The prevalences of HT in SLE, RA, SSc, MCTD, SS and PM/DM were 90-, 160-, 220-, 556-, 176- and 69-fold higher than in the general population, respectively. The prevalences of GD in the same systemic diseases were 68-, 50-, 102-, 76-, 74- and 37-fold higher than in the general population, respectively. Among all thyroid patients, 30% had associated systemic disease. In particular, 51% of HT and only 16% of GD subjects had any of the systemic disorders. MCTD, SS, SLE, RA, SSc and PM/DM were all more common among HT patients (20, 17, 7, 4, 2 and 2%, respectively) than in GD individuals (2, 5, 5, 1, 2 and 1%, respectively). CONCLUSION: Systemic and thyroid autoimmune diseases often overlap with each other. HT and GD may be most common among MCTD, SSc and SS patients. On the other hand, these systemic diseases are often present in HT subjects. Therefore it is clinically important to screen patients with systemic autoimmune diseases for the co-existence of thyroid disorders.
Arthritis, Rheumatoid↗
Prostate cancer underlying acute, definitive dermatomyositis: successful treatment with radical perineal prostatectomy.
The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation leading to progressive weakness of the proximal muscles. In 7-66% of cases of adult dermatomyositis different malignant tumours can promote the difficult cascade mechanisms at the cell level, leading to rapid weakness of skeletal muscles [1]. We report on a patient with all characteristic signs of acute, severe dermatomyositis associated with a low-grade, low-stage prostate cancer cured by radical perineal prostatectomy.
Adenocarcinoma↗
Paraneoplastic rheumatic syndromes.
Paraneoplastic symptoms caused by a malignancy but not directly related to tumour invasion are the result of a wide variety of tumour-derived biologic mediators, such as hormones, peptides, antibodies, cytotoxic lymphocytes, autocrine and paracrine mediators. Recognition of paraneoplastic syndromes is important, as it may lead to an early diagnosis of cancer. On the other hand, the clinical severity of the symptoms can be used as a guide to the extent of response to underlying tumour therapy. The quality of life of the patient is affected, therefore the palliative treatment of paraneoplasia is very important.
Humans↗
Evaluation of oral manifestations and masticatory force in patients with polymyositis and dermatomyositis.
BACKGROUND: The polymyositis (PM) and dermatomyositis (DM) complex encompasses a heterogeneous group of acquired autoimmune skeletal muscle diseases called idiopathic inflammatory myopathies (IIM). Despite their histological and immunopathological differences, the end result of the affected muscles in all of these entities is the triad of chronic inflammation, fibrosis and the loss of muscle fibres. The aim of this study was to perform a complete analysis of the orofacial abnormalities in 34 patients with PM and DM. METHODS: Evaluation of subjective oral symptoms, measurement of whole resting saliva flow rate (WS) with the 'spitting method', a visual investigation of the oral soft tissue alterations, light- and electron microscopic analysis of the symptoms of capillary abnormalities or signs of focal infiltration in labial biopsy specimens were carried out. The number of decayed, missing and filled teeth (DMF-T) according to the WHO recommendations (1997), the periodontal probing depth (PPD), the plaque index (PI; Silness-Löe) and the gingival index (GI; Löe-Silness) were determined. For comparison with healthy controls the masticatory force (MF) and the force of the upper extremities' flexors were measured with a specially developed device. The SPSS version 11.0 for Windows software program, two-tailed Student's t-test and Mann-Whitney test were used to statistically analyse all data. Values were considered to be significant if P level was < or = 0.05. RESULTS: Nine patients complained of subjective xerostomia, 11 showed the signs of salivary hypofunction (WS < or = 0.1 ml/min). The most prominent symptom of the oral mucosa and perioral tissues was the presence of telangiectasia, detected in seven cases. Fibrosis of the minor salivary glands was found in 12 patients, interstitial-perivascular infiltration was detected in eight cases, periductal infiltration in one case. The findings on dental and periodontal conditions indicate, that the patients with IIM diseases had significantly higher DMF-T scores (24.06 +/- 7.04 vs. 19.54 +/- 8.93, respectively; P = 0.002), they had less remaining teeth in average (15 vs. 20; P = 0.002), compared with the control group. Their oral hygiene was significantly worse (PI was 1.46 +/- 0.75 and 0.73 +/- 0.54, P = 0.001). GI was significantly higher in the patients (1.27 +/- 0.60 and 0.66 +/- 0.56, respectively, P = 0.0003), although we could not demonstrate any difference in the severity of the periodontal destruction between patients and controls. Masticatory force was significantly weaker in the first molar region on both sides in the patient group (309 +/- 213 N and 113 +/- 146 N, P = 0.0016 for the right side; 315 +/- 239 N and 123 +/- 76 N, P = 0.009 for the left side), but only the left hands showed to be weaker in the patient group (77 +/- 27 N and 59 +/- 20 N, P = 0.04). CONCLUSION: In conclusion, the present study showed that, patients with IIM diseases have hyposalivation and mucosal alterations, mainly in the form of telangiectasia. They have increased prevalence of dental caries, which may be secondary to the hyposalivation. The masticatory and the upper extremity flexor forces indicate that the weakness of the masticatory muscles can manifest earlier than that of the arms.
Adult↗
Detection of TT virus in patients with idiopathic inflammatory myopathies.
The TT virus, a recently identified single-stranded DNA virus with unknown pathogenicity, has been shown to commonly infect humans. Viruses have been considered to contribute to disease pathogenesis in autoimmune disorders including idiopathic inflammatory myopathies (IIMs) and rheumatoid arthritis (RA). We assessed the prevalence of TTV infection in IIM compared with that in patients with RA and healthy blood donors. Detection of TTV was conducted by nested PCR and real-time PCR in the sera of 94 patients with IIM, 95 RA patients. and 95 age- and sex-matched healthy blood donors. Identity of the PCR products was confirmed by sequencing. TTV DNA was detected in 61 of 94 (64.9%) patients with IIM, in 64 of 95 (67.4%) patients with RA, and in 62 of 95 (65.3%; P > 0.05) healthy individuals. Age, sex, activity, or duration of disease had no influence on TTV positivity in either group. However, patients with severe IIM (n = 36) had a significantly higher rate of TTV infection (31/36, 86.1%) than patients with mild disease (30/58, 51.7%, P < 0.05, chi(2) = 10.0). Disease was considered severe in IIM when immunosuppressive treatment was necessary because of continuous high activity and/or serious inner-organ involvement despite corticosteroid treatment. In conclusion, although we found the detection rate of TTV similar in patients with idiopathic inflammatory myopathies and rheumatoid arthritis and comparable to that in healthy controls, our data suggest that infection with TT virus may result in a more severe disease in patients with idiopathic inflammatory myopathies.
Adult↗
Cancer-associated myositis: clinical features and prognostic signs.
Idiopathic inflammatory myositis is characterized by progressive weakness of the proximal muscles. There is a higher risk of malignancy than in the normal population. The aim of this study was to evaluate the frequency of malignancy among 251 myositis patients. We also compared clinical and immunological characteristics of cancer-associated myositis with primary myositis. There were no malignancies among polymyositis, overlap, or juvenile myositis patients. Twenty-two of ninety dermatomyositis patients also had a malignant disease. Patients with cancer-associated dermatomyositis were significantly older than primary myositis patients and had more severe cutaneous and muscle symptoms. Dysphagia and diaphragmatic involvement were more frequent among cancer-associated patients, while extramuscular features were less frequent. After successful treatment of the malignancy, we were able to manage myositis symptoms. One-year survival rate was significantly better in primary dermatomyositis patients. The subset of cancer-associated myositis differs from primary myositis in many aspects of its clinical and immunological features. Prognosis and life expectancy in cancer-associated myositis patients is determined by the underlying malignant disease. Therefore, age- and sex-specific examinations for detection of an underlying malignancy are important in the management of patients with dermatomyositis.
Adult↗
Antiphospholipid and antisynthetase syndrome in a patient with polymyositis-rheumatoid arthritis overlap.
Idiopathic inflammatory myopathies, mainly polymyositis (PM) may occur in the course of several autoimmune diseases. The overlapping forms of myositis, when the patient also meets the criteria for rheumatoid arthritis (RA), affect 3%-5% of myositis patients [1]. To the best of our knowledge this is the first report on the overlapping form of RA, antiphospholipid syndrome (APS) and the serological subgroup of PM, the antisynthetase syndrome (ASS).
Adult↗
Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases.
The idiopathic inflammatory myopathies are characterized by chronic muscle inflammation and involvement of internal organs, which contribute considerably to the morbidity and mortality of the disease. We conducted the current study to determine the survival data for patients with idiopathic inflammatory myopathies according to the presence of extramuscular clinical manifestations. We also determined the cumulative survival probability and the long-term prognosis and analyzed the causes of death at a single clinical immunology center.A survival analysis was performed using data for 162 patients diagnosed between 1976 and 1997 according to Bohan and Peter's criteria. Patients were followed up for a minimum of 5 years (median, 101.5 mo) or to date of death. Cumulative survival probability was calculated by the Kaplan-Meier method. The influence of extraskeletal and extramuscular involvement was analyzed as prognostic factors for death by Cox proportional hazards survival model. Eighteen disease-specific deaths occurred; pulmonary and cardiac complications were the most frequent causes of death. Global survival rates were 95%, 92%, and 89% for 1, 5, and 10 years, respectively. Analysis for clinicopathologic subgroups revealed that cancer-associated myositis had the worst prognosis, while juvenile and overlap myositis had the best prognosis. Five- and 10-year survival rates were 94.2% and 89.4% for patients with primary polymyositis and 90.1% and 86.4% for primary dermatomyositis patients, respectively. In the whole group of patients with idiopathic inflammatory myopathy, cardiac (p < 0.01) and respiratory muscle involvement (p = 0.045) were significant prognostic factors for death. In the group of patients with primary polymyositis/dermatomyositis, cardiac involvement was the main prognostic factor for death (p < 0.01). Myositis patients described in this study have higher survival rates than reported previously worldwide. We examine the reasons for the differences between the data in the current study and the available survival data in the relevant literature.
Adult↗
[Clinical characteristics of juvenile dermatomyositis].
INTRODUCTION: Dermatomyositis, belonging to the group of the idiopathic inflammatory myopathies, is characterized by bimodal pattern of age-specific incidence of rates, with peaks in age group from 5 to 14 years (juvenile dermatomyositis) and in age group from 45 to 64 years (adult dermatomyositis). THE AIM OF THIS STUDY: Is to evaluate the clinical characteristics of 12 patients with juvenile dermatomyositis followed by the 3rd Department of Internal Medicine, University of Debrecen and the 2nd Department of Pediatrics, Semmelweis University, Budapest. METHODS: The authors analyzed the medical records of the patients with juvenile and adult form of dermatomyositis retrospectively. RESULTS: All of the children had symmetrical weakness of the proximal muscles. The most frequent cutaneous features were facial erythema and Gottron papules (11/12). The extramuscular manifestations were also assessed. 7 children had arthralgia. There were observed pulmonary fibrosis, Raynaud-syndrome, dysphagia and sicca-syndrome in the same patient, whose disease is overlapped with progressive systemic sclerosis. In view of the clinical course, the authors found that prevalence of polycyclic (relapsing-remitting) and monophasic subtypes of the disease were similar (6/12 and 5/12). Finally, all of the patients achieved remission, however, 2 patients have to take low-dose corticosteroid therapy permanently to maintain remission. One patient's cutaneous symptoms proved to be persistent and in further 2 cases, regression of the calcinosis is slow, but continuous. DISCUSSION: The authors compare their data of juvenile patients with the data of the relevant literature and to their experience with the management of adult DM patients. It seems to be reasonable to treat the patients in centres.
Adolescent↗
[Polymyositis/scleroderma autoantibody-positive scleroderma with dermatomyositis (scleromyositis)].
Polymiositis/scleroderma autoantibody positive scleroderma with dermatomyositis connected to interstitial lung fibrosis is reported. The recently observed overlap syndrome is characterised by the presence of special autoantibodies, HLA-type association and benign course. A new skin symptom (mechanic's hands) is described which may indicate the syndrome and one of its important internal manifestations, the interstitial lung disease.
Autoantibodies↗
[Myositis associated with tumors].
AIMS OF THE STUDY: In the 214 polymyositis / dermatomyositis patients in our department we studied the association of polymyositis / dermatomyositis with malignant tumors, the clinical specificities and therapeutic response changes in these cases. METHODS: Retrospective analysis of the data available since 1985 of the patients treated at the DEOEC 3(rd) Department of Internal Medicine. RESULTS: From 60 patients with dermatomyositis 9 were diagnosed with neoplasia. In 5 patients simultaneously with the dermatomyositis diagnosis, in the other 4 patients 2-2-3-7 years after the onset of dermatomyositis. In the 154 patients with polymyositis we did not observe development of tumors. CONCLUSIONS: Neoplasm was observed in 15% of patients with dermatomyositis. The patients presented with serious skin involvement which did not respond well to the dermatomyositis treatment. The patients in whom tumors developed simultaneously with the dermatomyositis required more aggressive treatment. After the therapy of the tumor (surgery, radiotherapy), the skin and muscle symptoms responded better to the immunosuppressive therapy.
Aged↗
[Amyopathic dermatomyositis].
Dermatomyositis is a systemic autoimmune disease which belongs to the group of idiopathic inflammatory myopathies. The disease is rare with an incidence of 0.1-1/100,000 and a prevalence of 1-6/100,000. Women are affected twice as often as men. In some patients the disease presents with dermatologic changes weeks to months before the myopathy arises. It was observed that in some patients the myositis develops much later and sometimes not at all. Therefore, the term of dermatomyositis sine myositis used in earlier literature has been changed to amyopathic dermatomyositis. The most important question is whether the patient needs systemic therapy with its possible side effects yet possibly preventing the appearance of myositis or only local therapy for the skin manifestations is necessary. The goal of this article is to summarize the latest findings in amyopathic dermatomyositis.
Dermatomyositis↗
Rare, late complications in a patient with Hodgkin's disease.
We report a unique case of a cured Hodgkin's disease patient who developed several complications decades after mantle and neck radiation therapy. First on the right, then years later on the left side of the neck a pharyngocutaneous fistula developed complicated by rupture of the carotid arteries on both sides necessitating their ligation. The fistula on the right side was successfully closed by plastic surgery, but the one on the left side is still a problem as severe hypoperfusion of the brain limits therapeutic possibilities. We discuss the relationships between the treatment for Hodgkin's disease and subsequent complications as well as treatment of these complications.
Adult↗