Hypertension and nephrology

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

HARIS Ágnes, POLNER Kálmán

SEPTEMBER 10, 2017

Hypertension and nephrology - 2017;21(04)

[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.]

COMMENTS

0 comments

Further articles in this publication

Hypertension and nephrology

[Thought about renovascular hypertension by a special case report]

GAJDÁN Nikolett, LÉGRÁDY Péter, BAJCSI Dóra, MORVAY Zita, NAGY Endre, LETOHA Annamária, KYPROS Constantinou, FEJES Imola, SONKODI Sándor, ÁBRAHÁM György

[Renovascular hypertension is a well-known form of secunder hypertension. Two thirds of cases are caused by atherosclerotic plaque and one third are caused by fibromuscular dysplasia. The prevalence of it is less than 1%. Digital subtraction angiography is considered the goldstandard diagnostic method. The 58-year old female patient was hospitalized with resistant hypertension. Duplex ultrasonography showed fibromuscular stenosis the in left renal artery. Percutaneous transluminal angioplasty and stenting were performed. Her blood pressure normalized. The patient did not attend the control examinations. Next time in 2001, she was referred to our emergency department with increased blood pressure of 210/140 mmHg. Following control ultrasonography angiography showed total occlusion of the left renal artery and significant stenosis of the right renal artery. Left nephrectomy was necessary due to shrunken kidney and dilatation and stenting of the right renal artery. The blood pressure normalized again. Since 2004 until 2014 despite of the regular visits, we detected in stent restenosis of the right renal artery almost in each year. Even so, renal function was preserved all the time. In autumn of 2014, the patient suffered severe stroke, and few months later at the age of 74 she died. There are many open questions to discus concerning the right treatment of renovascular hypertension yet. Even so by performing 12 intravascular interventions we could ensure her acceptable quality of life for 16 years.]

Hypertension and nephrology

[A Letter to Our Readers]

Hypertension and nephrology

[Isolated systolic hypertension in the elderly and very elderly]

FARSANG Csaba

[In the elderly (˃65 yrs) and very elderly (˃80 yrs) large clinical investigations showed that isolated systolic hypertension is the most frequent form of hypertension. In the background, several cardiovascular, neural and hormonal changes have been proved. One of the most important pathogenetic factor is the increase of arterial stiffness. This leads to the increase of pulse wave velocity and systolic blood pressure, and also to the decrease of diastolic blood pressure. Consequently, pulse pressure increases. All these factors contribute to the increase in incidence and prevalence of cardiovascular consequences of hypertension, which are more frequent than in younger ages.]

Hypertension and nephrology

[The Frequency of Isolated Systolic Hypertension in the Elderly among the Hungarian Hypertension Population ]

KISS István, PAKSY András, KÉKES Ede

Hypertension and nephrology

[Sex-specific clinical and exercise based risk assessment of the total mortality risk]

KÉKES Ede

[In both sexes combining different types clinical questionnaire and results of exercise test in a point system can more reliably predict 10 years mortality or survival. The method in both sexes is reliably suitable for the screening of highly endangered individuals in everyday practice.]

All articles in the issue

Related contents

Clinical Neuroscience

[Treatment and new evidences in neuromyelitis optica spectrum disorder ]

ILLÉS Zsolt

[Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9–10):309–321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.]

Clinical Neuroscience

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

[Efficiency and safety of the vaccination against H1N1 influenza virus in inflammatory bowel disease]

FARKAS Klaudia, JANKOVICS István, MELLES Márta, NAGY Ferenc, SZEPES Zoltán, WITTMANN Tibor, MOLNÁR Tamás

[INTRODUCTION - Inactivated influenza and H1N1 vaccination is recommended yearly for patients with inflammatory bowel disease receiving immunosuppressive therapy; however, immunomodulator and biological therapy might impair the immune response to the vaccination. In our study, we assessed whether immunity can develop in response to H1N1 influenza vaccination in patients receiving immunomodulator and/or biological therapy. We also assessed the occurrence of side effects after the immunisation in these patients. PATIENTS AND METHODS - In our prospective study, blood samples were obtained from 24 patients (12 Crohn’sdisease, 12 ulcerative colitis) one month after immunisation against influenza A/California/ 07/2009 (H1N1) virus. At the time of vaccination, all patients have been receiving immunomodulator and/or biological therapy for at least three month. Antiviral antibodies were detected by using microneutralisation assay. The safety of the vaccination was assessed by questionnaires. RESULTS - Every patient developed complete immunity against influenza A (H1N1) virus, independently from the type of immunosuppressive therapy. Regarding side effects, local symptoms occurred in six patients and systemic symptoms in another six patients. Mild diarrhea occurred in five patients. Moderate exacerbation of the disease was observed in 2 patients with Crohn’s disease and in one patient with ulcerative colitis. CONCLUSIONS - According to our results, immunocompromised patients with IBD can be safely advised to receive the vaccination. In our study, all patients developed adequate immunity according to microneutralisation titers.]

Hypertension and nephrology

[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.]

Lege Artis Medicinae

[Rituximab therapy in rheumatoid arthritis]

SÜTŐ Gábor

[Rheumatoid arthritis is a chronic, lifelong disease that causes severe joint deformity, reduces quality of life, and, if not treated appopriately, leads to disability and substantial premature mortality. Its treatment is a multistep procedure, where different grades of treatment options follow each other. Besides traditional, diseasemodifying antirheumatic drugs (DMARDs) and biological therapies inhibiting TNF, a new therapautic option is the use of a chimeric antibody, rituximab, which inhibits B lymphocyte function. This drug is an effective and safe choice for those patients who have received various anti- TNF therapies or do not tolerate TNF inhibition.]