18 F-FDG PET/CT in Pediatric Immunoglobulin G4-Related Disease.

ABSTRACT: A 14-year-old boy presented with severe, nonintentional weight loss, fatigue, and cough. Investigations were notable for raised inflammatory markers and significantly elevated serum immunoglobulin G4 (IgG4). 18 F-FDG PET/CT was obtained for assessment of disease involvement and extent, to exclude differential diagnoses and to guide biopsy. The study demonstrated extensive FDG-avid nodal disease and multisystem organ involvement. Excisional biopsy of a right inguinal lymph node demonstrated reactive changes with numerous IgG4-positive plasma cells consistent with IgG4-related disease. He was commenced on immunosuppressive therapy with consequent weight restoration and symptom resolution.

Survey of parent and carer experiences and expectations of paediatric rheumatology care in New South Wales.

Objective The aim of the present survey of parent and carers was to document the level of care and services currently provided to children diagnosed with rheumatic diseases (RD) in New South Wales (NSW), Australia. Methods The survey included parents and carers of children presenting to paediatric rheumatology (PR) services in NSW. Subjects attending PR clinics in both public and private settings were invited to participate in an online or paper survey. Results Overall, 148 surveys were completed. The process of obtaining the diagnosis of RD was described as being 'difficult' or 'very difficult' by 56.1% (n=83) of the surveyed cohort, and 41.2% (n=61) saw four or more different clinicians before diagnosis. Between symptom onset and final diagnosis, 42.6% (n=63) of participants reported a delay of 5 months or more, and 16.9% (n=25) waited longer than 12 months. Eventually, 91% (n=134) were referred to a paediatric rheumatologist and 63.5% (n=94) were seen within 4 weeks from initial referral. More than half the respondents felt that general practitioners (GPs) and general paediatricians were not aware of RD. Overall, respondents felt that improved knowledge of PR diseases among GPs, improved access to PR clinics, improved educational materials for patients and families, access to speciality rheumatology nurses and coordinated rheumatology teams would have significantly improved the experience of their child's disease. Conclusions Children with RD in NSW still experience significant delays from symptom onset to final diagnosis through consultations with multiple healthcare professionals. Multidisciplinary team care was not the norm for this patient group, despite established national and international management standards. What is known about the topic? Early diagnosis and management by a multidisciplinary team is the gold standard in PR management. Delays in diagnosis may significantly impair the outcomes of children diagnosed with RD, with reduced quality of life, increased pain level and worse long-term prognosis. What does this paper add? Children diagnosed with RD in NSW endure significant delays from symptom onset until a final diagnosis is made, with multiple consultations with different healthcare professionals. When the referral to PR services in NSW is made, RD children are mostly seen within 4 weeks, faster than other international standards. GPs and paediatric rheumatologists in NSW helped improve the children's and their family's experience of the diagnosis and treatment of a rheumatic condition and better informed them using appropriate educational materials. What are the implications for practitioners? This paper provides new evidence to practitioners to increase their knowledge of the current experiences and expectation of the paediatric rheumatology care in NSW.