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Lege Artis Medicinae

JULY 20, 2019

[Severe polymyositis associated with multiplex pulmonary abscesses]

SZABÓ Katalin, VINCZE Anett, NAGY-VINCZE Melinda, DANKÓ Katalin, GRIGER Zoltán

[INTRODUCTION – Idiopathic inflammatory myopathies are heterogeneous autoimmune diseases characterized by immune mediated inflammation of the skeletal muscles. CASE REPORT – A case of a 62-year-old male patient with severe proximal muscle weakness, elevated creatine kinase and swallowing difficulity is presented. Electromyography showed myogenic pattern, thus probable polymyositis was diagnosed. Radiological examination has confirmed bilateral multiplex lung lesions, which were caused by the possibility of tumor, tuberculosis, vasculitis and abscess as well. The condition of the patient deteriorated, nasogastric feeding, high dose steroid treatment was initiated, which reduced the patient's creatinine kinase values, but muscle strength was not changed. Based on the results of various investigations, the condition of the patient was finally confirmed by the development of myositis, resulting dysphagia, chronic aspiration, and multiplex lung abscess. Antibiotic therapy, steroid treatment was continued and finally intravenous immunoglobulin treatment was administered. The condition of the patient gradually improved, the swallowing dysfunction disappeared, and the lung abscesses were resolved. As a result of physiotherapy and rehabilitation treatment, the patient could walk again. CONCLUSIONS – Nasogastric feeding is recommended to prevent aspiration in the case of myositis-associated dysphagia. In case of steroid refractory therapy, the use of intravenous immunoglobulin may be effective. ]

Clinical Neuroscience

JANUARY 30, 2015

[Inclusion body myositis]

BODOKI Levente, VINCZE Melinda, GRIGER Zoltán, CSONKA Tamás, DANKÓ Katalin, HORTOBÁGYI Tibor

[The idiopathic inflammatory myopathies are systemic, chronic autoimmune diseases characterized by proximal symmetrical muscle weakness. One of the main diseases in this group is inclusion body myositis (IBM), an underdiagnosed, progressive muscle disease characteristically affecting the middle-aged and older population. It has a slow, relentlessly progressive course. The precise pathogenesis of the disease remains unknown. In most of the cases it is diagnosed a few years after the appearance of the first symptoms. The muscle biopsy typically shows endomysial inflammation, with invasion of mononuclear cells into the non-necrotic fibers, and also rimmed vacuoles. It appers, that both inflammation and degeneration are present at the onset of the disease. Our aim is to raise awareness about this disease which leads to severe disability, with clinicopathological case presentations and literature overview, emphasizing the importance of collaboration between the clinician and the neuropathologist. No effective therapy is currently available but the rapid diagnosis is essential to slow disease progression. Although this is a relatively rare disease, patients are presenting not only in immunology outpatient clinics; our reports aims to raise awareness and facilitate accurate early diagnosis of IBM. ]

Clinical Neuroscience

SEPTEMBER 30, 2014

[Anti-signal recognition particle autoantibody positive myopathy]

BODOKI Levente, VINCZE Melinda, HORTOBÁGYI Tibor, GRIGER Zoltán, CSONKA Tamás, DANKÓ Katalin

[The idiopathic inflammatory myopathies are systemic, autoimmune diseases characterized by proximal symmetrical muscle weakness. We review the myositis-associated and myositis-specific autoantibodies, among them the anti- SRP autoantibody. Among those autoimmune myopathy cases, that are associated with autoantibodies, we can detect anti-SRP autoantibody positive myositis cases. We describe the role of signal recognition particle, also its structure and role in protein biosynthesis. We review how the necrotizing autoimmune myopathy is identified, what kind of differences are presented in relationship with the classical polymyositis cases. As we will see the anti-SRP titer correlates with the activity of the disease. We serve some examples from the literature how the disease looks in the childhood and also some rare cases from the literature. At the end of our review we present a case report to draw attention to the importance of the disease.]

Clinical Neuroscience

MARCH 30, 2013

[Inclusion body myositis - a rarely recognized disorder]

DÉZSI Lívia, DANIELSSON Olof, GÁTI István, VARGA Edina, VÉCSEI László

[Inclusion body myositis is the most common disabling inflammatory myopathy in the elderly. It is more frequent in men and after the age of 50 years. Inflammatory and degenerative features coexist. There is a T-cell mediated autoimmunity driven by in situ clonally expanded cytotoxic CD8-positive T-cells invading non-necrotic muscle fibres expressing MHC-I antigen. The hallmarks of degeneration are the deposition of protein aggregates and the formation of vesicles. The course of the disease is slow and the diagnosis is usually set after several years. The muscle weakness and wasting is assymetric, affecting predominantly distal muscles of the upper extremity and proximal muscles of the legs. The signs and clinical course can be characteristic, but the diagnosis is established by muscle biopsy. There is currently no evidence based effective treatment for sIBM. Prednisone, azathioprine, methotrexate, cyclosporine and IFN-β failed. Oxandrolon did not improve symptoms. Treatment with intravenous immunglobuline (IVIG) induced in some patients a transient improvement of swallowing and of muscle strenght, but the overall study results were negative. A T-cell depleting monoclonal antibody (alemtuzumab), in a small uncontrolled study slowed down disease progression for a six-month period. Repeated muscle biopsies showed the reduction of T-cells in the muscle and the suppression of some degeneration associated molecules. An effective therapeutic mean should act on both aspects of the pathomechanism, on the inflammatory and the degenerative processes as well.]

Lege Artis Medicinae

FEBRUARY 22, 2013

[Necrotising autoimmune myopathy showing dermatomyositis symptoms during persistent statin treatment]

TIHANYI László, SÜTŐ Gábor, VERESS Gábor

[INTRODUCTION - Dermatomyositis is an idiopathic autoimmune disease. Diagnosis has to be confirmed by biopsy, because clinical symptoms can be similar to those in other myopathies. CASE REPORT - The authors describe the development of dermatomyositis in a 59- year-old man. The patient had a heart attack in 1988. He had been taking simvastatin for three years and atorvastatin since 2002. In June 2008 he complained of myalgia and weakness, became physically very inactive but visited our specialist only half a year later. Laboratory examinations showed a substantial increase of CK, CKMB, LDH and HBDH with troponin T positivity (troponin I was negative). A subsequent biopsy confirmed dermatomyositis. Treatment with azatioprine and methylprednisolone and suspending statin therapy resulted in the regression of his clinical symptoms, his muscles gradually became stronger and his laboratory values normalised. CONCLUSION - During statin treatment in 5% of the cases myopathy can occur, which might develop into amore severe inflammatory disorder.]

Lege Artis Medicinae

FEBRUARY 21, 2004

[ACUTE DERMATOMYOSITIS ASSOCIATED TO THE CARCINOMA OF THE PROSTATE]

TÁLLAI Béla, MORSHED Ali Salah, FLASKÓ Tibor, PONYI Andrea, DANKÓ Katalin, TÓTH Csaba

[INTRODUCTION - In some cases of polymyositis/ dermatomyositis (PM/DM) of autoimmune origin, different malignant tumours can initiate the difficult cascade mechanisms at cell level leading to the rapid weakness of the skeletal muscles. Till now, in the international literature only four cases of PM/DM associated with cancer of prostate has been reported. CASE REPORT - Authors present a case of a 57 yearold male patient, where weakness in patient's movements developed leading to total immobility in 3 months. Purple discoloration developed on his hand and face. Significantly elevated creatin kinase (CK) levels and blood sediment rate with mild anaemia were observed during laboratory examinations. Dysphagia and lack of appetite resulted in the loss 10 kgs in body weight. Both clinical evaluation, elevated serum CK level, skin symptomes, positive electromyography and muscle biopsy confirmed acute definitive dermatomyositis. Urological examination revealed a palpable hard area at the right lobe of the prostate. Prostate biopsy confirmed the presence of carcinoma in the right lobe of the prostate. There was no sign or symptome referring to either local propagation or distant manifestation. Therefore, radical prostatectomy was performed, the tumorous prostate and both seminal vesicles were removed. Histological examination proved malignant focus in the right lobe of the prostate. After the operation patient gradually became stronger, corticosteroid medication were decreased then stopped. Patient's original muscle power and movement recovered and his previous body weight was regained. During the regular control examinations all results of laboratory tests are in normal range. CONCLUSION - In the background of some autoimmune diseases malignant tumours can be revealed. It is rare when urological neoplasms initiative the process. In men with PM/DM commencing beyond 50 years of age it is necessary to think of the presence of prostate cancer, which can be cured by performing radical operation in appropriate time.]

Hungarian Immunology

FEBRUARY 15, 2004

[Diagnostic value of MRI in patients with juvenile dermatomyositis]

CONSTANTIN Tamás, PONYI Andrea, BALÁZS György, SALLAI Ágnes, DANKÓ Katalin, FEKETE György, KARÁDI Zoltán

[Diagnosis of juvenile dermatomyositis is based on the presence of proximal muscle weakness, characteristic skin lesions, muscle enzyme elevation in the serum, and may requires the performance of invasive procedures such as electromyography and/or muscle biopsy. Magnetic resonance imaging (MRI) is considered to be an objective non-invasive tool to detect muscle involvement for diagnosis as well as for follow-up studies. We report a case of a 12 years old girl with definitive juvenile dermatomyositis. She received glucocorticoid therapy and achieved remission of the disease. After a long-term relapse free period, she was presented with severe proximal muscle weakness and normal creatinine kinase levels. The laboratory studies did not reveal acute inflammation or infection. In this case MRI was diagnostic to the relapse of juvenile dermatomyositis, with an increased STIR (short tau inversion recovery) signal of proximal muscles. The muscle involvement detected by MRI correlated with functional ability. After she achieved clinical remission, further follow-up MRI scans demonstrated that the affected muscles had returned to normal signal intensity. Findings of dermatomyositis on MRI scans include increased signal intensity in the affected muscles, perimuscular edema, chemical-shift artifact, and increased signal intensity in subcutaneous tissue. MRI is a sensitive technique and proposed to be a good indicator for an early diagnosis of the disease. MRI may also help to guide the muscle biopsy and may enhance the sensitivity of histological examination. After completion of therapy, MRI may be used for monitoring the progress of the disease as signal intensity of affected muscles returns to normal. MRI is also helpful, if the diagnosis is suspected but has not been formally evaluated.]

Hungarian Immunology

JANUARY 10, 2004

[Intracytoplasmic cytokines in dermatomyositis]

ALEKSZA Magdolna, SZEGEDI Andrea, ANTAL-SZALMÁS Péter, SIPKA Sándor, DANKÓ Katalin

[OBJECTIVE - To investigate the intracellular and soluble cytokine levels in peripheral blood of patients with active and inactive dermatomyositis (DM). Methods The frequency of the intracellular IFN-g, IL- 4 and IL-10 expression of CD4+ or CD8+ cells were determined by flow cytometry. We measured the concentrations of soluble cytokines with commercial ELISAs. RESULTS - In active DM we observed decreased IFNg expression of CD4+ and CD8+ cells. These prominent changes disappeared in the inactive stage of the disease. In DM we could not detect a significant change in the intracellular IL-4 cytokine expression either in the active or in the inactive form. The IL-10 expression was elevated in the inactive state of the patients. CONCLUSION - We can state that a difference between aDM and iDM seems to exist in the level of peripheral blood lymphocytes and their intracellular cytokine content.]

Hungarian Immunology

JUNE 20, 2002

[Ocular myositis]

KISS Emese, FACSKÓ Andrea, DÉVÉNYI Katalin, DANKÓ Katalin, ZEHER Margit

[INTRODUCTION - Dermato-/polymyositis is an autoimmune disorder, which belongs to the idiopathic inflammatory myopaties. It involves skeletal muscles in form of weakness and inflammatory infiltrates. Characteristic skin lesions are present in dermatomyositis. Other organs may also be affected mainly in the presence of myositis specific autoantibodies. The inflammation usually involves the proximal muscles of extremities. CASE REPORT - In the present work we report the case of a 52-year-old woman. In the previous history the removal of rectal adenocarcinoma was remarkable in 1994. After that she received chemotherapy. She complied for severe headache and pain in the right eye in 2000 October, therefore a skull CT was performed, indicating thickening of rectus medalis muscle within orbital cavity. There was an enhancement of contrast material in the muscle. Glaucoma was excluded. Neurologist suspected the presence of myositis and indicated 0.5 mg/kg corticosteroid therapy. Soon after the left eye became painful, but due to the corticosteroid treatment both eyes became painless. A control orbital CT was completely negative in 2000 November. Immunology consultancy revealed a mild proximal muscle atrophy in both lower extremities, but CPK and LDH enzyme levels were normal, EMG was characteristic for mild chronic nerve lesion. The biopsy, taken from the involved proximal muscle of lower extremity, did not show inflammatory infiltration. Complete screening for cancer was negative. Thyroid gland disease could be excluded. Immune laboratory data were negative, autoantibodies, including anti-Jo1, could not be detected. Based on the results a rare disease, ocular myositis was diagnosed. Considering the clinical improvement, the withdrawal of corticosteroid therapy was offered. Stringent immunology and oncology follow-up is required. CONCLUSION - In relation to our case report, we discuss clinical symptoms of orbital myositis, diagnostic procedures to identify the disease and also differential diagnostic considerations.]

Hungarian Immunology

JUNE 20, 2002

[Anti-synthatase syndrome]

PONYI Andrea, CONSTANTIN Tamás, DANKÓ Katalin

[The idiopathic inflammatory myopathies (IIM) are autoimmune diseases, characterized by symmetric proximal muscle weakness. Over the last several decades, many abnormalities of the cellular and humaral immune systems of IIM patients have been described. Some of these are autoantibodies unique to the IIM (the myositis specific autoantibodies MSA). The MSAs are antigen-driven, arise months prior to the onset of myositis, correlate in titre with disease-activity. Studies in the recent years proved that clinically and immuno-geneticaly different disease entities can be defined using myositis specific auto-antibodies. Their use make the serological classification. Myositis specific autoantibodies make it possible to define more homologies subgroups within polymyositis/dermatomyositis that may support the adequate treatment. The most common MSA is the antihistidyl- transfer RNA synthetase (anti-Jo-1), which can be found in 5-30% of patients with myositis. Patients with anti-synthatase antibodies tend to have characteristic clinical presentation of fever, small joint arthritis, intestinal lung disease, Raynaud's phenomenon, mechanic's hands and severe myositis. Sera of 65 PM/DM were tested for anti-Jo- 1 antibody. 15 patients (23%) had anti-Jo-1 antibody (10 PM, 5 DM). The patients with anti-Jo- 1 antibody has a significantly higher incidence of interstitial lung disease, arthritis, fever and Raynaud's phenomenon. These patients needed not only corticosteoid therapy, but other immunosupressive treatment. All 15 patients presented with the onset of weakness between February and July. The determination of myositis-specific autoantibodies has produced more homogenous grouping within the polymyositis/dermatomyositis patients.]