The outcomes of patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) who are not treated are unclear. This study aimed to clarify these outcomes and identify the factors related to them. We retrospectively evaluated various clinical features including laboratory data and involved organs at diagnosis in 107 patients with IgG4-RD, who were followed up for more than 6 months, at a single center in Japan. We compared the clinical features of the 27 untreated patients with those of the 80 patients treated with glucocorticoid. The patient outcomes were investigated, and logistic regression analysis was performed to identify factors related to them. The patients comprised 73 men and 34 women (median age 67 years). The untreated patients had significantly lower IgG4-RD responder index (9 vs. 12) and fewer affected organs (1 vs. 3) than did those treated with glucocorticoid. Of these 27 patients, 8 experienced deterioration of IgG4-RD after the diagnosis. In the age- and sex-adjusted logistic regression analysis, serum IgG4 elevation (per 100 mg/dL, odds ratio 1.194, 95% confidence interval 1.017–1.402) was the only significant factor related to disease deterioration in untreated patients with IgG4-RD, whereas not serum IgG4 levels (per 100 mg/dL, odds ratio 0.995, 95% confidence interval 0.921–1.075) but history of allergy (OR 3.134, 95% confidence interval 1.094–8.977, P = 0.033) related to deterioration in patients who underwent treatment. Serum IgG4 levels may be a useful predictor of unfavorable outcomes in untreated patients with IgG4-RD, who tend to have fewer affected organs and lower IgG4-RD responder index.

Dialysis disequilibrium syndrome (DDS) is a neurological complication that has been known to occur after hemodialysis (HD). In recent years, the prevalence of DDS has been low as the symptoms are widely recognized; hence, preventive therapies, such as the slow and gentle procedure for HD, are often administered before starting dialysis. However, once DDS occurs, it may cause seizures, coma, and even death in severe cases. Since there has been no established treatment, recognizing risk factors and preventing the syndrome is important. A 76-year-old man was admitted to our hospital due to exacerbation of chronic heart failure. He also had a history of chronic kidney disease and had consulted with his home doctor about the preparation for HD a month before admission. After treatment with diuretics, the symptoms ameliorated, but he experienced presyncope and malaise. Laboratory tests revealed acute anemia and a decrease in renal function. Upper gastrointestinal endoscopy revealed active bleeding from a gastric ulcer, which was successfully stopped. However, his consciousness deteriorated because of uremia; hence, HD was initiated. We used a cellulose triacetate membrane with a surface area of 1.3 m 2 and maintained a dialysate flow rate of 500 ml/min with a blood flow rate of 120 ml/min. Four hours after starting HD, he suddenly developed generalized tonic convulsions. The dialysis was immediately stopped, and the patient was transferred to an intensive care unit. A computed tomography scan of the head showed mild edematous change of the brain, and laboratory tests also revealed a rapid decrease of urea nitrogen. We rationalized that he might have developed DDS. After injection of levetiracetam for the treatment of seizures, we initiated continuous hemodiafiltration as renal replacement therapy. Fortunately, his consciousness gradually improved, and he was completely alert on day 18 after admission. With reference to our current report, DDS can occur even following acute kidney injury, as the progression rate of the injury and accumulation of blood urea may not correlate with the risk of the syndrome.

Objectives In IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), involvement of two or more sets of lacrimal glands (LGs) and/or major salivary glands (MSGs) is regarded as a specific finding with diagnostic significance. This study aimed to clarify the influence of this factor on the overall clinical picture of IgG4-DS. Methods We retrospectively reviewed the medical records of 130 patients with IgG4-related disease, 97 of whom were diagnosed with IgG4-DS. We determined their clinical features according to the presence/absence of involvement of ≥2 sets of LGs and/or MSGs and compared the results with those obtained in 33 DS-limited patients. Results The IgG4-DS patients comprised 60 men and 37 women (median age 65 years). The median serum IgG4 level at diagnosis was 548 mg/dL. The patients with involvement of ≥2 sets (n = 44) had significantly more affected organs, lower serum C3 and C4 levels, and a tendency to have higher serum IgG levels and IgG4-RD responder index than did those without it (n = 53). In the 33 DS-limited patients, these two groups had no significant differences in clinical features. Conclusions Involvement of ≥2 sets of LGs and/or MSGs suggests greater systemic disease activity mainly reflected by involvement of more organs.

BackgroundIgG4-related periaortitis/periarteritis (IgG4-PA) is a major organ manifestation of IgG4-related disease (IgG4-RD) [1]. In the affected aorta/artery, arteriosclerotic changes are frequently observed [2]. However, the influence of arteriosclerosis on IgG4-PA and aneurysmal changes of the affected lesions has not been well evaluated.ObjectivesThis study aimed to clarify the relationship of arteriosclerosis with periaortic/periarterial lesions and their aneurysmal changes.MethodsWe retrospectively investigated the medical data, including the presence of IgG4-PA at diagnosis, new development of IgG4-PA during the clinical course, arteriosclerotic and aneurysmal changes of the affected lesions, and classic risk factors for arteriosclerosis in 130 patients with IgG4-RD at a single center in Japan. The relationship of arteriosclerosis with the periaortic/periarterial lesions and their aneurysmal changes was statistically analyzed.ResultsOf the 130 patients with IgG4-RD, 44 comprising 39 men and 5 women (mean age 67.4 years) were diagnosed with IgG4-PA. The mean follow-up period was 72.8 months, and the mean serum IgG4 level at diagnosis was 851 mg/dL. The proportions of males (88.6% vs. 55.8%, P<0.001), positive smoking history (81.4% vs. 47.6%, P<0.001), and history of malignancy (36.4% vs. 14.0%, P=0.006) were significantly higher in the 44 patients with IgG4-PA than in the 86 without it. Arterial wall calcification and/or mural thrombus in the affected lesions were detected in 86.0% of the IgG4-PA patients, some of whom had had these arteriosclerotic changes before the development of periaortic/periarterial lesions. Of the 44 with IgG4-PA, 9 had aneurysmal changes of the affected lesions at the time of the first diagnosis of IgG4-RD. Notably, these 9 patients with aneurysmal changes had a significantly higher incidence of hypertension (77.8% vs. 34.3%, P=0.027) and mural thrombus in the affected lesions (100% vs. 64.7%, P=0.044) than the remaining 35 without aneurysmal changes. However, diabetes mellitus, dyslipidemia, smoking history, C-reactive protein level elevation, or arterial wall calcification of the affected lesions did not differ significantly between the two groups.ConclusionThe present study suggests that arteriosclerotic changes and the classic risk factors for arteriosclerosis may be related to periaortic/periarterial lesions and their aneurysmal changes in IgG4-PA.References[1] Stone JH, et al. IgG4-related disease. N Engl J Med. 2012 Feb 9;366(6):539-51.[2] Inoue D, et al. Immunoglobulin G4-related periaortitis and periarteritis: CT findings in 17 patients. Radiology. 2011 Nov;261(2):625-33.Disclosure of InterestsNone declared

Objectives Although elevated serum IgA levels are thought to exclude a diagnosis of IgG4-related disease (IgG4-RD), IgG4-RD has been definitively diagnosed in some patients despite elevated serum IgA levels. This study aimed to clarify the prevalence of elevated IgA levels in patients with IgG4-RD and to compare the clinical features of IgG4-RD patients with and without elevated IgA levels. Methods The clinical features of 169 IgG4-RD patients were retrospectively compared among those with and without elevated serum IgA levels. Results Of the 169 patients with IgG4-RD, 17 (10.1%) had elevated serum IgA levels. Those with elevated serum IgA levels showed higher serum CRP levels and lower prevalence of relapse than those without. Other clinical features did not differ significantly, including inclusion scores of the ACR/EULAR classification criteria. Cox regression analysis showed that elevated serum IgA levels were associated with a lower incidence of relapse. Moreover, patients with elevated serum IgA levels showed prompt improvement in response to glucocorticoids in the IgG4-RD responder index. Conclusion Some patients diagnosed with IgG4-RD have high serum IgA levels. These patients may form a subgroup, characterized by good response to glucocorticoids, less frequent relapse, mildly elevated serum CRP levels, and possible complications of autoimmune diseases.