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Clinical Neuroscience

NOVEMBER 20, 2015

[A rare complication of a rare disease; stroke due to relapsing polychondritis]

KILIC COBAN Eda, XANMEMMEDOV Elimir, COLAK Melek, SOYSAL Aysun

[Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of cartilaginous structures. Its diagnosis is based primarily on clinical features such as laboratory parameters, biopsy. Neurological complications occur in 3% of the cases and are classified as an important cause of death. The cranial nerve disorders are most common but hemiplegia, ataxia, myelitis, polyneuritis, seizures, confusion, hallucination and headache can also happen. The aetiology of central nervous system involvement is still unknown. Moreover stroke has rarely reported in these patients. The diagnosis of stroke is challenging because of its rarity among these patients. Perhaps vasculitis is the common underlying mechanism. Also meningitis and encephalitis can occur during the course of RP. A 44 year-old woman was admitted with uncontemplated left hemiparesis, redness, swelling, and tenderness of the metacarpophalangeal and interphalangeal joints of the right hand and the cartilaginous portion. White blood cell count, C-reactive protein and the erythrocyte sedimentation rate were elevated. Vasculitis biomarkers were normal in our patient. Carotid and vertebral artery doppler ultrasonography, cranial and cervical MR Angiography were normal. Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed. Hemiparesis was her first neurological manifestation that led her to doctors for diagnosis. Our patient fulfilled the criteria of RP so no biopsy was needed. She was treated with oral prednisolone (80 mg/day) and aspirin (300 mg/day) and now she is on 10 mg prednisolone and 150 mg azathioprine. Two months later her physical and neurological symptoms returned to normal.]

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. ]

Hypertension and nephrology

OCTOBER 20, 2018

[Hungarian Vasculitis Registry – results of the first five years]

HARIS Ágnes, TISLÉR András, ONDRIK Zoltán, FILE Ibolya, MÁTYUS János, ZSARGÓ Eszter, DEÁK György, AMBRUS Csaba

[Launching the Hungarian Vasculitis Registry aimed to collect information about prevalence and outcome of our patients with ANCA-associated vasculitis, and treatment protocols of the disease. The on-line data collection has been developing dynamically since its initiation five years ago, presently 278 patients’ files are available. Patients’ mean age is 58.2±14.5 years, 62% are women; their disease is associated with c-ANCA positivity in 51% and p-ANCA in 49%. At diagnosis GFR was 24.6±21.6 ml/min/1,73 m2, that time 29%, during the total follow up 39% of the registered subjects needed dialysis. Renal replacement therapy could be discontinued in 23% of them. In cases with focal histological changes, also with upper respiratory tract and skin involvement dialysis was significantly less frequently necessary, which underlines the importance of early diagnosis. In induction therapy steroid was administered for 94% of the patients, 85% of them got cyclophosphamide, 59% was treated by plasmapheresis, 11% got rituximab. Maintenance treat ment contained steroid in 80%, per os cyclophosphamide in 23%, parenteral cyclophosphamide in 22%, furthermore 40% of the patients got azathioprin, 8 subjects got mycophenolate and 6 got methotrexate. Median follow up was 30 months (IQR 6-78), during which period 20% of the patients died, 5% got kidney transplantation, and 5% were lost to follow up. Median survival was 14.8 years, five years survival was 85%, and ten years survival was 70%. Long term survival in patients with c-ANCA vasculitis seemed better comparing to p-ANCA vasculitis, but when correcting by age this difference disappeared. Predictors of death were age and dialysis dependent renal failure. Relapses developed in 27% of patients, 28% of them presented in the first year, 21% suffered it after five years of care. Collected data by the Hungarian Vasculitis Registry shows our society’s successful professional activity. Our results are comparable to the published data in the literature, yet there are several areas in our care where further improvements are warranted in order to increase our patient’s survival and quality of life.]

Hypertension and nephrology

SEPTEMBER 10, 2017

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 2]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]

Lege Artis Medicinae

JULY 20, 2017

[Eosinophil granulomatous polyangiitis]

WITTMANN Zsófia, KARDOS Magdolna, FINTHA Attila, KERKOVITS Lóránt, AMBRUS Csaba

[Due to the high prevalence of kidney involvement, patients with systemic autoimmune disorders, also including small vessel vasculitides are frequently seen in neph-rology centers. Activated neutrophils attacking the wall of various blood vessel are key features in these diseases, leading to bleeding, occlusion, ischaemia and tissue necrosis. This latter finding is reflected in the term necrotising vasculitis. In this paper, we present a case of eosinophil ganulomatous polyangiitis (EGPA, formerly called Churg-Strauss syndrome), the least common form of ANCA associated small vessel vasculitides. We found it very interesting but not uncommon that our patient was admitted to the nephrology ward with vague symptoms that became more and more suggestive and typical for vasculitis during our observation, guiding us to the right diagnosis. Timely and appropriate immunosuppressive therapy based on immunology lab report and histology findings resulted in good response and remission of the disease in our patient. ]

Hypertension and nephrology

MAY 20, 2017

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 1]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy, both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]

Lege Artis Medicinae

NOVEMBER 03, 2015

[Sudden death of a patient with purpura - post mortem recognized eosinophilic granulomatosis with polyangiitis]

DOBREAN Noémi, HAJNAL-PAPP Rozália, TUSA Magdolna, OROJÁN Iván, CSERNI Gábor

[INTRODUCTION - Systemic diseases may sometimes be challenging because physicians do not think about synthesizing the parts to a single entity. CASE REPORT - A 49-year-old asthmatic female was admitted to hospital for the investigation of her cutaneous symptoms suggestive of vasculitis associated with diffuse joint complaints. The chest X-ray raised the possibility of pneumonia or neoplastic disease. Following an episode of chest pain relieved by a non-steroidal anti-inflammatory drug, she suddenly died. Her previous history included restrictive cardiomyopathy, insufficiency of both atrioventricular valves and long dating eosinophilia. Autopsy revealed a partly granulomatous eosinophilic inflammatory process in several organs, including the heart, the lungs, the kidneys, the colon and the pituitary gland. Retrospective collection of unknown anamnestic features and symptoms made possible to unify the pieces of information and symptoms to a single entity, the Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis, EGPA). CONCLUSIONS - Bronchial asthma seldom leads to death. It can rarely be part of the Churg-Strauss syndrome, of which the manifestation may be related to the administration of leukotriene antagonists also used in the presented case. These drugs may allow the withdrawal of systemic steroid therapy which is beneficial not only in the treatment of asthma but also of the syndrome. Lowering the dose of steroids may promote the development of the full blown pattern of the latter.]

LAM Extra for General Practicioners

DECEMBER 05, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]

Lege Artis Medicinae

SEPTEMBER 21, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]

Hypertension and nephrology

FEBRUARY 20, 2014

[Prognostic value of histopatologic classification in ANCA-association vasculitis]

FILE Ibolya, BIDIGA László, TRINN Csilla, UJHELYI László, BALLA József, MÁTYUS János

[Introduction: Rapidly progressive glomerulonephritis is life-threatening manifestation of antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), often diagnosed only in advanced stage of renal failure. In 2010 a new histopathologic classification of ANCA- associated vasculitis was published by an international working group of nephropathologists. Vasculitis cases were classified in four groups: focal, crescent, mixed and sclerotic. Method: The aim of our study was to re-evaluate the predictive value of this new classification regarding renal outcome. From the 88 patients with ANCA-associated vasculitis treated in our department from 1996 to 2013, 39 were involved. Results: By retrospective evaluation of biopsy samples, patients were classified as 11 in focal, 12 in crescent, eight in mixed, eight in sclerotic group. There was no significant difference among the four groups regarding the mean age, sex, ANCAtype and initial eGFR. Due to the treatment, the eGFR values significantly increased in the focal and in the crescent groups. Eleven patients needed dialysis at presentation and three of them recovered, none of them belonged to the sclerotic group. The cumulative renal response to treatment was 100% in the focal, 87.5% in the mixed, 64% in the crescent and 62% in the sclerotic group. Renal response at one year treatment was 80%, thirty-one of the thirty-nine patients were dialysis independent. All patients were alive at one year, by year five two patients from the sclerotic group died. Conclusion: The new nephropathological classification of AAV is useful in predicting the renal response to treatment. Nephropathology can optimize the system by mentioning the specific percentage of normal glomeruli in the biopsy specimen.]