Incidence of ANCA antibodies in patients with systemic lupus erythematosus (SLE) is described in 24-31 %, but they are not related to the distribution and severity of organ involvement in SLE; the routine monitoring is not recommended. Overlap syndrome of systemic lupus erythematosus and ANCA associated vasculitis (AAV) is rare. The difficult diagnosis and treatment of this syndrome is described in this case report of the patient with SLE and severe kidney involvement resulting from AAV.

• Klíčová slova
• ANCA associated vasculitis, overlap syndrome, systemic lupus erythematosus.,
• MeSH
• ANCA-asociované vaskulitidy * komplikace   MeSH
• incidence MeSH
• lidé MeSH
• syndrom MeSH
• systémový lupus erythematodes * komplikace   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

OBJECTIVES: The RITUXVAS trial reported similar remission induction rates and safety between rituximab and cyclophosphamide based regimens for antineutrophil cytoplasm antibody (ANCA)-associated vasculitis at 12 months; however, immunosuppression maintenance requirements and longer-term outcomes after rituximab in ANCA-associated renal vasculitis are unknown. METHODS: Forty-four patients with newly diagnosed ANCA-associated vasculitis and renal involvement were randomised, 3:1, to glucocorticoids plus either rituximab (375 mg/m(2)/week×4) with two intravenous cyclophosphamide pulses (n=33, rituximab group), or intravenous cyclophosphamide for 3-6 months followed by azathioprine (n=11, control group). RESULTS: The primary end point at 24 months was a composite of death, end-stage renal disease and relapse, which occurred in 14/33 in the rituximab group (42%) and 4/11 in the control group (36%) (p=1.00). After remission induction treatment all patients in the rituximab group achieved complete B cell depletion and during subsequent follow-up, 23/33 (70%) had B cell return. Relapses occurred in seven in the rituximab group (21%) and two in the control group (18%) (p=1.00). All relapses in the rituximab group occurred after B cell return. CONCLUSIONS: At 24 months, rates of the composite outcome of death, end-stage renal disease and relapse did not differ between groups. In the rituximab group, B cell return was associated with relapse. TRIAL REGISTRATION NUMBER: ISRCTN28528813.

• Klíčová slova
• B cells, Cyclophosphamide, Granulomatosis with polyangiitis, Systemic vasculitis, Treatment,
• MeSH
• ANCA-asociované vaskulitidy komplikace   farmakoterapie   imunologie   MeSH
• azathioprin terapeutické užití   MeSH
• B-lymfocyty cytologie   MeSH
• chronická renální insuficience farmakoterapie   etiologie   imunologie   MeSH
• chronické selhání ledvin etiologie   MeSH
• cyklofosfamid terapeutické užití   MeSH
• glukokortikoidy terapeutické užití   MeSH
• granulomatóza s polyangiitidou komplikace   farmakoterapie   imunologie   MeSH
• imunosupresiva terapeutické užití   MeSH
• kombinovaná farmakoterapie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mikroskopická polyangiitida komplikace   farmakoterapie   imunologie   MeSH
• myší monoklonální protilátky terapeutické užití   MeSH
• počet lymfocytů MeSH
• přežití po terapii bez příznaků nemoci MeSH
• progrese nemoci MeSH
• rituximab MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• randomizované kontrolované studie MeSH
• srovnávací studie MeSH
• Názvy látek
• azathioprin MeSH
• cyklofosfamid MeSH
• glukokortikoidy MeSH
• imunosupresiva MeSH
• myší monoklonální protilátky MeSH
• rituximab MeSH

We present a case of a patient with systemic vasculitis suffering--besides heart, skin and gastrointestinal lesions--from the rarely reported involvement of the central nervous system. Even though the diagnosis could not be ascertained precisely, immunosuppressive therapy led to prompt regression of symptoms including initially present neurologic manifestations.

• MeSH
• centrální nervový systém patologie   MeSH
• imunosupresiva terapeutické užití   MeSH
• lidé středního věku MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• systémová vaskulitida farmakoterapie   patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH
• Názvy látek
• imunosupresiva MeSH

Secondary vasculitides usually accompany various common and rare conditions, Their clinical picture is very diverse, they can be loclaized or genaralized. Most frequently, we find parainfectious, drug-related and paraneoplastic vasculitides, less commonly in connective tissue diseases, after radiotherapy or transplantation. Vasculitides may be associated to infection of any origin. Drug-related vasculitides are mainly confined to the skin with picture of leukocytoclastic angiitis but visceral organs may be involved too. Paraneoplastic vasculitides usually accompany solid tumours and lymphoproliferative processes. When related to connective tissue diseases we can observe vasculitis in rheumatoid arthritis, systemic lupus erythematosus, Sjoegren syndrome, systemic sclerosis and other conditions. The diagnosis of vasculitis is usually based on pathological findings from biopsy. Management lies in treatment of underlying disease and if it is ineffective combined immunosuppression should be introduced.

• Klíčová slova
• connective tissue diseases, drugs, infection, irradiation, neoplasms, vasculitis,
• MeSH
• kožní leukocytoklastická vaskulitida * MeSH
• lidé MeSH
• nemoci pojiva * komplikace   MeSH
• vaskulitida * diagnóza   etiologie   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Rare chronic childhood vasculitides lack a reliable disease activity assessment tool. With emerging new treatment modalities such a tool has become increasingly essential for both clinical practice and therapeutic trials to reproducibly quantify change in disease state. OBJECTIVE: To develop and validate a paediatric vasculitis activity assessment tool based on modification of the Birmingham Vasculitis Activity Score (BVASv.3). METHODS: A paediatric vasculitis registry was reviewed to identify clinical features missing in the BVASv.3. A modified nominal group technique was used to develop a working version of the Paediatric Vasculitis Activity Score (PVAS). Prospective validation provided tool reliability, reproducibility and responsiveness to change. Training of assessors was done according to the BVAS principles. RESULTS: BVAS items were redefined (n=22) and eight paediatric items added in Cutaneous (n=4), Cardiovascular (n=3) and Abdominal (n=1) sections. The final PVAS has 64 active items in nine categories. The principles of new/worse and persistently active disease were retained as were the overall score and weighting of categories. The median PVAS in 63 children with systemic vasculitis was 4/63 (0-38/63). There was a high interobserver agreement for the overall as well as for subsystem scores (linear-weighted-κ ≥0.87). PVAS correlated with physician's global assessment (p<0.01); treatment decision (p=<0.01) and erythrocyte sedimentation rate (ESR) (p=0.01). In response to treatment, 15/19 patients assessed demonstrated a significant fall in PVAS (p=0.002), with good agreement among assessors for this change. CONCLUSIONS: The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.

• Klíčová slova
• Disease Activity, Outcomes research, Systemic vasculitis,
• MeSH
• chronická nemoc MeSH
• dítě MeSH
• glukokortikoidy terapeutické užití   MeSH
• lidé MeSH
• mladiství MeSH
• odchylka pozorovatele MeSH
• předškolní dítě MeSH
• reprodukovatelnost výsledků MeSH
• stupeň závažnosti nemoci * MeSH
• vaskulitida diagnóza   farmakoterapie   MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• validační studie MeSH
• Názvy látek
• glukokortikoidy MeSH

ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting small vessels in various organ systems, accompanied by the presence of ANCA antibodies in serum. AAV represents a group of the most common systemic vasculitis in adulthood, and based on clinical manifestations this disease entity includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly Wegeners granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors, such as infections. The mechanisms by which ANCA antibodies cause vasculitis involves excessive neutrophil activation, that subsequently leads to release pro-inflammatory cytokines, reactive oxygen species and lytic enzymes. In addition, activated neutrophils induce the formation of neutrophil extracellular traps in a process called NETosis. The released neutrophil antigens are exposed to the immune system via antigen presenting cells, which further stimulates antibody production and creates a vicious circle with tissue destruction. Understanding the pathogenesis of AAV represents the key which provides not only optimal diagnosis and treatment, but also gives the pathologist a tool for deeper insight into the morphological features of disease progression, including the various stages of development and healing.

• Klíčová slova
• AAV, ANCA associated vasculitis, GPA, MPA, pathophysiology,
• MeSH
• ANCA-asociované vaskulitidy * patologie   MeSH
• Churgův-Straussové syndrom * MeSH
• dospělí MeSH
• granulomatóza s polyangiitidou * MeSH
• lidé MeSH
• mikroskopická polyangiitida * MeSH
• protilátky proti cytoplazmě neutrofilů MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• protilátky proti cytoplazmě neutrofilů MeSH

In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

• Klíčová slova
• Corticosteroids, Cyclophosphamide, Disease Activity, Systemic vasculitis, Treatment,
• MeSH
• ANCA-asociované vaskulitidy farmakoterapie   patologie   MeSH
• biopsie normy   MeSH
• imunosupresiva terapeutické užití   MeSH
• indukce remise metody   MeSH
• lidé MeSH
• management nemoci * MeSH
• opakovaná terapie metody   MeSH
• recidiva MeSH
• výměna plazmy MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• směrnice pro lékařskou praxi MeSH
• Názvy látek
• imunosupresiva MeSH

We present a case report of patient with systemic vasculitis with affection of heart, skin, gastrointestinal tract and rare involvement of central nervous system. Diagnosis of systemic vasculitis was based on clinical manifestations, blood hypereosinophilia and brain magnetic resonance imaging. Immunosuppressive therapy led to regression of symptoms including initially present neurologic manifestation.

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• paréza etiologie   MeSH
• systémová vaskulitida komplikace   diagnóza   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

The case presented describes a high-risk pregnancy of a woman with systemic lupus erythematosus (SLE) with multiple lesions of central nervous system (CNS), vasculitis, secondary epilepsy and antiphospholipid syndrome (APS). At gestational age 28 weeks and 3 days the pregnancy was urgently terminated via caesarean section and an extremely hypotrophic immature newborn with a birth weight of 580 g was born. The high disease activity in the mother at the time of conception and the histologically proven chronic placental insufficiency due to APS are presumably the causes for the extensive hypotrophy of the neonate. The significant comorbidity of the newborn, including respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, osteopathy of prematurity, transient hypothyroidism and hypocortisolism, vesicoureteral reflux, and hypertonic-hyperexcitation syndrome complicated his three-month stay in NICU. A positive titre of transplacentally transferred anticardiolipin and anti-β2 glycoprotein antibody was detected in the child and persisted through the following 30 months. During the three-year follow-up, significantly delayed neuropsychological development with microcephaly (-4 SD) and short stature of the child was observed. Finally, the authors discuss possible causes of neuropsychological consequences in children of mothers with SLE and APS and emphasize the need for long-term monitoring and specialized care to improve development of these children.

• Klíčová slova
• Systemic lupus erythematosus, cognitive disorder, hypotrophy, newborn, pregnancy, psychomotor development,
• MeSH
• antifosfolipidový syndrom komplikace   diagnóza   imunologie   MeSH
• časové faktory MeSH
• císařský řez MeSH
• dospělí MeSH
• epilepsie etiologie   MeSH
• gestační stáří MeSH
• komplikace těhotenství diagnóza   etiologie   imunologie   terapie   MeSH
• lidé MeSH
• novorozenec nedonošený * imunologie   MeSH
• novorozenec s extrémně nízkou porodní hmotností * imunologie   MeSH
• novorozenec MeSH
• porodní hmotnost MeSH
• prognóza MeSH
• systémový lupus erythematodes komplikace   diagnóza   imunologie   MeSH
• těhotenství MeSH
• vaskulitida centrálního nervového systému komplikace   MeSH
• vývoj dítěte MeSH
• vývojové poruchy u dětí etiologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Hepcidin is a key regulator of iron metabolism and plays an important role in many pathologies. It is increased by iron administration and by inflammation, while erythropoiesis downregulates its expression. It decreases iron availability and thus contributes to anemia of chronic diseases. The aim of the study was to measure hepcidin as a marker and pathogenetic factor in ANCA-associated vasculitis (AAV). Hepcidin plasma concentration was measured by the immunological method in 59 patients with AAV and compared to patients with non-vasculitic etiology of chronic kidney disease, patients on hemodialysis (HD), with systemic lupus erythematodes (SLE) and to healthy controls and blood donors, and was correlated with the parameters of iron metabolism, inflammation, activity of the process and kidney function. Hepcidin concentration was increased in patients with AAV, SLE and HD and correlated positively with C-reactive protein, serum ferritin and creatinine, and negatively with hemoglobin and serum transferrin. In active form of AAV it correlated with the clinical scoring system (BVAS). Hepcidin can thus be considered as a pathogenetic factor of anemia in AAV and can be used for evaluation of inflammation in AAV and as an additional marker in active forms of the disease.

• MeSH
• ANCA-asociované vaskulitidy krev   diagnóza   MeSH
• anemie krev   diagnóza   MeSH
• biologické markery krev   MeSH
• chronická renální insuficience krev   diagnóza   MeSH
• dospělí MeSH
• hepcidiny krev   MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• systémový lupus erythematodes krev   diagnóza   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• biologické markery MeSH
• hepcidiny MeSH