[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.]

[Research results in recent years have demonstrated that B-lymphocytes play a crucial role in the pathogenesis of multiple sclerosis (MS). The increased understanding of the disease process has resulted in the development of B cell-targeting antibodies as potential drugs for both relapsing and progressive forms of MS. Therefore, B-cell depletion therapies are becoming more prominent and determining in reducing disease progression. The first B-cell depleting anti-CD20 monoclonal antibody was rituximab, which has also been studied in MS and, following favourable results, new drugs have been developed with a similar point of attack. In 2017, the FDA and in 2018, the EMA approved ocrelizumab, another anti-CD20 monoclonal antibody, for the treatment of relapsing-remitting (RRMS) and primary progressive multiple sclerosis (PPMS). This was a particularly significant advance in the treatment of PPMS, as it was the first medication with a proven effect of reducing progression in PPMS. Ofatumumab, a fully human anti-CD20 monoclonal antibody, has emerged recently as a new player in B-cell depletion therapy. The drug has also recently been approved by the EMA in March 2021 for use in relapsing forms of MS. In this review, we detail the mechanism of action and efficacy of anti-CD20 therapies currently used in MS. ]

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.

[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.]

[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.]