Article Text
Statistics from Altmetric.com
3.1 Analysis of Raynaud's phenomenon in an infant and teenage population
Objectives—To analyse the onset and evolution patterns as well as the prognostic value of Raynaud's phenomenon (RP) in a cohort of children diagnosed and followed up in our paediatric rheumatology service.
Material and methods—In a prospective study several clinical, analytical, and outcome variables were collected from a group of patients under the age of 18 years who were admitted to our department because of a history of positive RP.
Results—54 patients (47 female, 7 male) with a mean age of onset of the disease of 13.5 years (range 2–18) were studied. In 31 patients (57%) RP occurred in isolation and in 23 patients (43%) it coexisted with arthritis, subcutaneous nodules and/or hand oedema.
After an average evolution period of 7 years (range 1–14) 10 patients (19%) developed a defined connective tissue disease (CTD) (4 mixed connective tissue diseases, 3 scleroderma, 2 systemic lupus erythematosus, 1 juvenile chronic arthritis), 31 patients (57%) presented some feature of undifferentiated CTD (arthritis, hand oedema, nodules, finger ischaemia, livedo, mucose ulcers, iridocyclitis, positive antinuclear antibodies (ANA), and pathological capillaroscopy pattern). 13 patients (24%) remain currently as primary RP.
Conclusions—(1) Over 50% (75% in our case) of patients with paediatric onset RP, eventually develop a CTD or present features consistent with this kind of disorder.
(2) The greater proportion found in our study might be explained by the selection of patients examined.
(3) Arthritis, hand oedema, co-occurrence of ANA and the presence of findings in nail capillaroscopy, have a good prognostic value.
3.2 Severe cardiac disease among children with diffuse cutaneous systemic sclerosis (SSc) and polymyositis: report of 4 cases
Patients and methods—We retrospectively reviewed the charts of 4 girls who met the ACR criteria for diffuse cutaneous SSc and had polymyositis, as defined by the presence of proximal muscle weakness and raised serum creatine phosphokinase level. Disease duration and age on admission ranged from 4 to 12 months …