The two most common entities among generally rare but under-diagnosed autoinflammatory bone disorders are chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, ...hyperostosis, and osteitis (SAPHO) syndrome. Due to their similarities, many authors consider CRMO to be a subtype of SAPHO syndrome. The aim of this study was to compare clinical, laboratory, and imaging features and outcomes of patients with CRMO and SAPHO. The analysis of the data from 6 children with CRMO (four girls and two boys, age 3.5-14 years) and of 6 children (6 boys, age 13.5-17.5 years) with SAPHO syndrome was performed. The initiating symptoms in all patients with CRMO were bone pain with multifocal bone lesions. There were no skin manifestations. Five out of six patients achieved control with nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, while one patient required disease-modifying antirheumatic drugs (DMARDs). The initiating symptom in five patients with SAPHO syndrome were severe acne, while in one patient acne occurred two years after the disease onset. Two patients typically developed inflamed sternoclavicular joints and sternum, while the others showed changes affecting other skeletal regions. Three patients achieved control with NSAIDs and corticosteroids, the others required DMARDs and TNFα inhibitors. In comparison with patients with CRMO, patients with SAPHO suffered more frequent and longer lasting exacerbations. In conclusion, CRMO and SAPHO syndrome have an array of common characteristics, but also a number of differences. Nevertheless, further investigation into the etiopathogenesis is required to establish a definite relationship between CRMO and SAPHO.
Vaskulitisi su rijetke reumatske bolesti nepoznate etiologije
kojima je osnovno obilježje nekrotizirajuća upala krvnih
žila. Posebnu skupinu čine granulomatozni vaskulitisi,
izrazito rijetko ...opisivani u dječjoj dobi. Prikazujemo dvije
bolesnice s Takayasuovim arteritisom (TA) kao entitetske
oblike rijetkih reumatskih bolesti. Jedna je bolesnica imala
TA tip II. a, a druga tip IV. U prve bolesnice nalazimo teške
simptome opstrukcijskih lezija aortnih ogranaka, osobito
tešku stenozu koronarnih arterija i okluziju lijeve potključne
arterije te stenozu torakalne aorte ispod istmusa. Bolest
je dijagnosticirana u akutnoj fazi, liječena je opsežno medikamentozno
(glukokortikoidi, citostatici, metotreksat) i
složenim kardiokirurškim pristupom, a zbog recidiva korištena
je i biološka (rituksimab) terapija. Druga je bolesnica
otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome
nađen je u teškom suženju aorte od dijafragme do bifurkacije
femoralnih arterija (mid aortic sindrom). U trenutku
dijagnoze sama bolest nije bila u aktivnoj fazi. Liječena je
osobitim kardiokirurškim pristupom i polimedikamentno
zbog recidiva. Obje su bolesnice u adolescentnoj dobi i
uspješno se liječe uz zadovoljavajuću kvalitetu života. Tip
II. a s dodatnom okluzijom koronarnih krvnih žila nije
prikazan u dostupnoj literaturi. Opisani vaskulitisi još uvijek
snažno povezuju pedijatrijsku kardiologiju i reumatologiju,
a prikaz svjedoči o važnosti timskog rada pedijatrijskih
kardiologa i reumatologa.
Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterized by the formation of antibodies against nuclear ...antigens. Vasculitis may be found in SLE, but it scarcely complies with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) criteria. We report five cases of severe JSLE associated with AAV diagnosed between 1991 and 2013 in three university-based tertiary care centers. The patients (3 girls and 2 boys, aged 12 to 17) presented with a severe clinical picture and the following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2), and gastrointestinal bleeding (n=1).All patients were proteinase 3 (PR3) ANCA positive, while two of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. They were treated with corticosteroids and immunosuppressive drugs. Remission of the disease was achieved in three patients. The course of the disease was worsening in two patients and we included rituximab (anti-CD20) in therapy. All of our patients presented as the most severe SLE patients, who must be diagnosed as soon as possible and treated very intensively. Since the comorbidity of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment, and the course of the diseases are experiences that can be of great help.
