Lupus nephritis in Croatian children: clinicopathologic findings and outcome.

We report clinical and histopathological features, treatment and outcome of 37 Croatian children with biopsy-proven lupus nephritis seen over a 30-year period. The mean age at lupus nephritis presentation was 12.11 ± 2.59 years (range 4.66-17.0). The most frequent histopathological finding was class IV (37.8%), followed by class III (35.1%), class V (16.2 %) and class II (10.8 %) lupus nephritis. Compared with other classes there were more boys among patients with class IV lupus nephritis, and hypertension, nephrotic syndrome and decreased estimated glomerular filtration rate at presentation were more common. The median histopathological activity and total scores were highest in class IV lupus nephritis patients. The mean follow-up was 7.14 ± 4.71 years, ranging from 1.1 years to 21.0 years. Kaplan-Meier estimate S: of patient and kidney (without renal failure) survival rate S: were 90.5% and 87 % at five years. The renal survival rate of class IV lupus nephritis patients was found significantly lower compared with other histological classes combined. Decreased estimated glomerular filtration rate at the time of diagnosis, class IV lupus nephritis versus other lupus nephritis classes, and high total histological score were the parameters significantly associated with adverse outcome. The therapy with cyclophosphamide showed as superior to the therapy with azathioprine.

Retrospective study of juvenile spondylarthropathies in Croatia over the last 11 years.

OBJECTIVES: To determine the frequency of juvenile spondylarthropathies (JSpA) among other rheumatic diseases in a pediatric clinic population in an 11-year period in Croatia and to review their clinical, epidemiological, radiographic and laboratory. METHODS: Of the 1264 patients with rheumatic diseases seen at a pediatric rheumatology center, 103 (8.2%) were diagnosed as having JSpA (56 boys, mean age 13.1 years, range 4.4-17.8 years), following the strict criteria of the European Spondylarthropathy Study Group. Medical history, clinical laboratory and imaging data of the 103 patients with JSpA were analyzed. RESULTS: Eighty-two (79.6%) patients had undifferentiated spondylarthropathy, 6 (5.8%) patients had reactive arthritis/Reiter's disease, 6 (5.8%) had arthritis associated with inflammatory bowel disease, 5 (4.9%) had psoriatic arthritis, and only 4 (3.9%) patients had ankylosing spondylitis. The most common symptoms at the disease onset in patients with JSpA were peripheral and axial arthritis, followed by enthesitis. A significant increase in the number of patients with axial arthritis, peripheral arthritis, ocular symptoms and enthesitis was found during mean period of follow-up of 6.45 years. HLA-B27 was present in 78 (75.7%) patients. CONCLUSION: In our hospital population the frequency of JSpA among other rheumatic disease was 8.2%. The disease was equally distributed among male and female patients, with onset around the age of 13 years. Most of the patients were diagnosed with undifferentiated spondylarthropathy.

Intracardiac juvenile xanthogranuloma in a newborn.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.