OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. METHODS: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. RESULTS: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively). CONCLUSION: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.

• MeSH
• Behcet Syndrome classification   diagnosis   MeSH
• Churg-Strauss Syndrome diagnosis   classification   MeSH
• Child MeSH
• Granulomatosis with Polyangiitis * classification   diagnosis   MeSH
• IgA Vasculitis diagnosis   classification   MeSH
• Humans MeSH
• Microscopic Polyangiitis classification   diagnosis   MeSH
• Adolescent MeSH
• Polyarteritis Nodosa classification   diagnosis   MeSH
• Predictive Value of Tests MeSH
• Child, Preschool MeSH
• Retrospective Studies MeSH
• Rheumatology standards   MeSH
• Sensitivity and Specificity * MeSH
• Takayasu Arteritis * classification   diagnosis   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Comparative Study MeSH
• Geographicals
• Europe MeSH

Předkládáme vám kazuistiku pacienta s klinickými příznaky připomínajícími syndrom kaudy, nicméně s negativním nálezem na zobrazovacích vyšetřeních. Pro silnou bolest a progredující slabost končetin byla provedena série vyšetření, která vedla k podezření na dermatomyozitidu. I přes intenzivní péči a kauzální terapii kortikoidy stav pacienta skončil exitem. Postmortem byly pozorovány histopatologické změny charakteristické pro polyarteritis nodosa. Polyarteritis nodosa je vzácným onemocněním, které může být opomíjeno v rámci diferenciální diagnostiky zánětlivých myopatií.

We report a case of a patient with clinical symptoms resembling cauda equina syndrome, however, with a negative finding on imaging studies. Due to severe pain and progressive limb weakness, a series of examinations were performed based on which dermatomyositis was suspected. Despite intensive care and causal treatment with corticoids, the patient expired. Histopathological changes characteristic for polyarteritis nodosa were observed postmortem. Polyarteritis nodosa is a rare disease which may be neglected in the differential diagnosis for inflammatory myopathies.

• MeSH
• Diagnostic Errors MeSH
• Fatal Outcome MeSH
• Humans MeSH
• Livedo Reticularis diagnosis   MeSH
• Methylprednisolone therapeutic use   MeSH
• Myositis diagnosis   etiology   MeSH
• Critical Care MeSH
• Polyarteritis Nodosa * diagnosis   MeSH
• Aged MeSH
• Cauda Equina Syndrome * diagnosis   MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Aged MeSH
• Publication type
• Case Reports MeSH

• MeSH
• Spondylitis, Ankylosing diagnostic imaging   complications   physiopathology   MeSH
• Behcet Syndrome complications   MeSH
• Cogan Syndrome complications   MeSH
• Diagnosis, Differential MeSH
• Hypertensive Retinopathy drug therapy   MeSH
• Arthritis, Juvenile diagnosis   complications   therapy   MeSH
• Keratitis diagnosis   drug therapy   MeSH
• Humans MeSH
• Eye Manifestations MeSH
• Polyarteritis Nodosa complications   MeSH
• Uveitis, Anterior epidemiology   etiology   therapy   MeSH
• Arthritis, Psoriatic diagnosis   drug therapy   complications   MeSH
• Arthritis, Reactive diagnosis   drug therapy   complications   MeSH
• Retinal Arterial Macroaneurysm MeSH
• Arthritis, Rheumatoid diagnosis   complications   therapy   MeSH
• Sjogren's Syndrome diagnosis   complications   therapy   MeSH
• Scleritis MeSH
• Keratoconjunctivitis Sicca diagnosis   therapy   MeSH
• Dry Eye Syndromes drug therapy   physiopathology   MeSH
• Scleroderma, Systemic complications   physiopathology   therapy   MeSH
• Lupus Erythematosus, Systemic diagnosis   drug therapy   complications   physiopathology   MeSH
• Takayasu Arteritis diagnosis   complications   physiopathology   MeSH
• Uveitis drug therapy   classification   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

OBJECTIVE: Cyclophosphamide (CYC) is used in clinical practice off-label for the induction of remission in childhood polyarteritis nodosa (PAN). Mycophenolate mofetil (MMF) might offer a less toxic alternative. This study was undertaken to explore the relative effectiveness of CYC and MMF treatment in a randomized controlled trial (RCT). METHODS: This was an international, open-label, Bayesian RCT to investigate the relative effectiveness of CYC and MMF for remission induction in childhood PAN. Eleven patients with newly diagnosed childhood PAN were randomized (1:1) to receive MMF or intravenous CYC; all patients received the same glucocorticoid regimen. The primary end point was remission within 6 months while compliant with glucocorticoid taper. Bayesian distributions for remission rates were established a priori for MMF and CYC by experienced clinicians and updated to posterior distributions on trial completion. RESULTS: Baseline disease activity and features were similar between the 2 treatment groups. The primary end point was met in 4 of 6 patients (67%) in the MMF group and 4 of 5 patients (80%) in the CYC group. Time to remission was shorter in the MMF group compared to the CYC group (median 7.1 weeks versus 17.6 weeks). No relapses occurred in either group within 18 months. Two serious infections were found to be likely linked to MMF treatment. Physical and psychosocial quality-of-life scores were superior in the MMF group compared to the CYC group at 6 months and 18 months. Combining the prior expert opinion with results from the present study provided posterior estimates of remission of 71% for MMF (90% credibility interval [90% CrI] 51, 83) and 75% for CYC (90% CrI 57, 86). CONCLUSION: The present results, taken together with prior opinion, indicate that rates of remission induction in childhood PAN are similar with MMF treatment and CYC treatment, and MMF treatment might be associated with better health-related quality of life than CYC treatment.

• MeSH
• Cyclophosphamide therapeutic use   MeSH
• Child MeSH
• Immunosuppressive Agents therapeutic use   MeSH
• Remission Induction methods   MeSH
• Mycophenolic Acid therapeutic use   MeSH
• Humans MeSH
• Adolescent MeSH
• Polyarteritis Nodosa drug therapy   MeSH
• Child, Preschool MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Equivalence Trial MeSH
• Research Support, Non-U.S. Gov't MeSH

• MeSH
• Anti-Bacterial Agents therapeutic use   MeSH
• Cyclophosphamide therapeutic use   MeSH
• Diagnosis, Differential MeSH
• Child MeSH
• Echocardiography MeSH
• Gastrointestinal Diseases MeSH
• Adrenal Cortex Hormones therapeutic use   MeSH
• Hypertension MeSH
• Humans MeSH
• Polyarteritis Nodosa * diagnosis   drug therapy   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

Polyarteritis nodosa (PAN) je vaskulitida arterií středního kalibru s pestrým klinickým obrazem. Mezi projevy PAN patří příznaky, jako je únava, horečka, hubnutí, kožní projevy, nespecifické artritidy, artralgie, postižení gastrointestinálního traktu či ledvin. PAN postihuje cévy středního kalibru, nezpůsobuje glomerulonefritídu a nepostihuje malé arterioly, kapiláry a venuly. Postižení velkých cév je vzácné. Popisujeme případ doposud zdravého 44letého muže, u kterého se choroba manifestovala kromě přetrvávajících febrilií i bolestmi břicha a hlavy, vysokým krevním tlakem. Při vyšetření počítačovou tomografií (CT) byl zjištěn nález infarktů obou ledvin, vpravo postihujících až 50 % parenchymu a dále byla zjištěna i disekce a. iliaca communis vlevo. Doplněné angiografické vyšetření viscerálních tepen prokázalo tři aneurysmata jaterních tepen, dále byly popsány v povodí arteria mesenterica superior a na jejunálních větvích segmentální ektázie. Diagnóza PAN byla stanovena na základě angiografického nálezu. Diagnózu podporoval klinický obraz s elevací dusíkatých katabolitů, myalgiemi, a vysokým krevním tlakem. Angiograficky byla také zjištěna disekce levostranné arteria iliaca communis a externa, která byla po vyloučení aterosklerotického či infekčního postižení hodnocena jako projev PAN. Postižení iliackých arterií je raritní manifestací PAN, v literatuře popisované jen kazuisticky. Přesto je třeba na ni pomýšlet. Je třeba věnovat pozornost tomuto možnému projevu a provádět zobrazovací vyšetření minimálně před angiografií, aby místo invaze do cévy nebylo zasažené.

Polyarteritis nodosa (PAN) is a medium-size-artery vasculitis with a varied clinical picture. The manifestations of PAN include symptoms such as fatigue, fever, weight loss, skin involvement, non-specific arthritis, arthralgia, gastrointestinal tract or kidney involvement. PAN affects the medium-size vessels, does not cause glomerulonephritis, and does not affect small arterioles, capillaries, and venules. The involvement of large blood vessels is rare. Herein, we describe a case of a healthy 44-year-old man who has been suffering from persistently elevated temperature, abdominal and head pain, and high blood pressure. Computer tomography (CT) examination revealed infarcts of both kidneys, affecting up to 50% of the parenchyma of the right kidney, and also a dissection of the left a. iliaca communis. Angiographic examination of the visceral arteries revealed three aneurysms of the hepatic artery, aneurysms in the superior mesenteric artery, and segmental ectasia in its jejunal branches. The diagnosis of PAN was based on angiographic findings and supported by a clinical picture with elevated nitrogen metabolites, myalgia, and high blood pressure. Angiography also revealed a dissection of the left a. iliaca communis and externa, which was evaluated as a manifestation of PAN after the exclusion of an atherosclerotic or infectious disease. The involvement of iliac arteries is a rare manifestation of PAN, described in the literature only as case-reports. Yet it is necessary to consider it. Attention should be paid to this possible manifestation and imaging should be performed at least before angiography to avoid penetration of the vessel at the site of vasculitic involvement.

• Keywords
• disekce arteria iliaca communis, aneurysma viscerálních tepen,
• MeSH
• Iliac Artery diagnostic imaging   pathology   MeSH
• Abdominal Pain etiology   MeSH
• Dissection MeSH
• Adult MeSH
• Fever etiology   MeSH
• Clinical Laboratory Techniques MeSH
• Kidney diagnostic imaging   pathology   MeSH
• Humans MeSH
• Young Adult MeSH
• Polyarteritis Nodosa * diagnosis   pathology   therapy   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Young Adult MeSH
• Publication type
• Case Reports MeSH

Prezentujeme případ pacienta s nově diagnostikovanou vaskulitídou typu polyarteritis nodosa, která se projevila makroskopickou hematurií, sekundární hypertenzí a vícečet-nými infarkty v obou ledvinách. Zobrazovací metody odhalily vícečetná drobná aneurys-mata renálních tepen, která způsobila okluzi segmentámích větví a následně ischemii částí parenchymu. Kromě postižení renálních tepen byla zjištěna i asymptomatická aneurysmata větví arteria hepatica propria a disekce levé arteria iliaca communis s přechodem na externu. Za zlatý standard obrazové diagnostiky polyarteritis nodosa je považována digitální subtrakčníangiografie, i když pokrok v CT angiografii může tuto metodu nahradit.

We present a case of a patient with a newly diagnosed vasculitis of polyarteritis nodosa, that manifested itself by macroscopic hematuria, secondary hypertension and multiple infarctions in both kidneys. Imaging methods revealed multiple small aneurysms of the renalarteries that caused occlusion of their segmental branches and consequently led to partialischemia of the parenchyma. Asymptomatic aneurysms on branches of proper hepatic artery and dissection of left common and extemaliliac artery were also diagnosed. Digital subtraction angiography is considered to be the gold standard of imaging in polyarteritis nodosa, although it could be replaced by CT angiography due to its rapid advances.

• MeSH
• Aneurysm diagnostic imaging   MeSH
• Angiography MeSH
• Hepatic Artery diagnostic imaging   pathology   MeSH
• Renal Artery diagnostic imaging   pathology   MeSH
• Computed Tomography Angiography MeSH
• Cyclophosphamide administration & dosage   MeSH
• Adult MeSH
• Humans MeSH
• Methylprednisolone administration & dosage   MeSH
• Polyarteritis Nodosa * diagnostic imaging   etiology   drug therapy   MeSH
• Prognosis MeSH
• Vasculitis diagnostic imaging   MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH

Termín "vaskulitida" označuje zánětlivé onemocnění cév vedoucí k destrukci cévní stěny, následné proliferaci a k jejich uzávěru. Podmínkou pro stanovení diagnózy je, aby cévní stěna byla primárním místem patologického procesu. Jako důsledek vznikají klinické syndromy na podkladě ischémie tkání zásobovaných poškozenými cévami a celkových projevů, které provázejí zánětlivé onemocnění. Vaskulitida může vzniknout de novo jako primární postižení cévní stěny neznámé etiologie nebo provázet jiné choroby. Diagnóza vaskulitidy je dána především histopatologickým nálezem z biopsie a někdy patologickými změnami, které prokážeme zobrazovacími metodami. Tento přehledný článek je věnován častějším primárním vaskulitidám, jejich diagnostice a léčbě.

By term "vasculitis" inflammatory disease of blood vessels is designated that leads to vessel wall destruction followed by proliferation and occlusion of their lumina. Basic condition for this diagnosis is that vessel wall is a primary site of the pathological process. Clinical syndromes are a consequence of this process resulting into ischaemia of tissues supplied by the affected vessels and with constitutional symptoms associated to the inflammatory disease. Vasculitis can occur de novo as a primary involvement of vessel wall of unknown aetiology or it develops secondary to other diseases. The diagnosis of vasculitis is usually based on pathological findings from biopsy or abnormalities detected by imaging methods. This review describes the common primary vasculitides, their diagnostics and management.

• MeSH
• Behcet Syndrome diagnosis   complications   pathology   MeSH
• Diagnosis, Differential MeSH
• Histological Techniques MeSH
• Humans MeSH
• Giant Cell Arteritis diagnosis   complications   pathology   MeSH
• Polyarteritis Nodosa diagnosis   pathology   MeSH
• Takayasu Arteritis diagnosis   complications   pathology   MeSH
• Vasculitis * diagnosis   classification   pathology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

• MeSH
• Angiography MeSH
• Cyclophosphamide administration & dosage   therapeutic use   MeSH
• Child MeSH
• Echocardiography methods   MeSH
• Brain Edema diagnostic imaging   MeSH
• Adrenal Cortex Hormones administration & dosage   therapeutic use   MeSH
• Administration, Intravenous MeSH
• Coronary Vessels diagnostic imaging   pathology   MeSH
• Skin pathology   MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Polyarteritis Nodosa diagnostic imaging   MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

• MeSH
• Humans MeSH
• Medication Therapy Management MeSH
• Polyarteritis Nodosa * drug therapy   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH