IgG4 disease with multiorgan involvement: a case report.

BACKGROUND: IgG4-related disease (IgG4RD) is a rare fibroinflammatory disease with multiorgan involvement. It presents insidiously over several years and can be a diagnostic enigma. Delays in diagnosis occur due to failure to consider IgG4 as a differential diagnosis, atypical presentations, and an insidious clinical course. CASE PRESENTATION: We report the case of a 70-year-old Sri Lankan man with pulmonary, renal, and neurological involvement of IgG4-related disease. Clinical manifestations evolved over a 4-year period and included exertional shortness of breath and dysesthesia of extremities. The diagnosis was established with clinical, radiological, and pathological criteria laid down by The American College of Rheumatology/European League Against Rheumatism in 2019. Following diagnosis, the patient was started on oral steroids, with rapid improvement of his respiratory and neurological symptoms. He is currently under follow-up and will be monitored with clinical and radiological parameters, complement levels, and lung function tests. CONCLUSION: This case outlines the presentation of a patient with IgG4-related disease with concurrent involvement of three uncommon sites. It highlights methods of diagnostic deduction by considering the clinical course of illness, imaging, and histopathology. It also describes evolving associations of IgG4-related disease with tuberculosis and lymphomas, which bear important diagnostic and therapeutic considerations.

Functional appliance treatment assessed using the PAR index.

The aims of this study were, first, to determine in children with Class II, division 1 malocclusions treated with functional appliances if, according to the Peer Assessment Rating (PAR) Index, lower incisor proclination affects the assessment of treatment outcome; and, second, to evaluate the effectiveness of such treatment before and after adjustment for any lower incisor proclination. Fifty-one children (32M, 19F; age and gender matched; 10 to 13 years) were randomly assigned to either an untreated group, one treated with Fränkel function regulators or one treated with Harvold activators. Study casts were assessed at the start, and after 6, 12, and 18 months of treatment/observation. The inclination of the upper and lower incisors was measured on lateral cephalometric radiographs taken at the start and 18 months later. There was wide variation in treatment response. The PAR scores decreased by more than 30% in 33% of the Fränkel group and by 75% in the Harvold group. When the score was adjusted to remove the effects of lower incisor proclination on the overjet, the treatment outcomes were "worse/no different" in 66% and "improved" in 33%, in both groups. It was concluded that functional appliances be used only on selected cases.