Background: Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disease. The major clinical features of SchS are urticarial rush and monoclonal gammopathy, accompanied by fever, joint pain and lymphadenopathy. There were few reports about SchS in Chinese population.Herein, we describe two patients with SchS in China and conducted a systematic literature review about SchS.Methods: Two Chinese Han patients were diagnosed as SchS in our department from 2017 to 2019.Their phenotype and genotype were carefully documented and studied. We also conducted a systematic literature review about SchS.Results: There were one man and one woman with an average disease-onset age of 52. Recurrent fever and urticarial rash occurred in both of them during the febrile attacks and normalized in asymptomatic intervals. Other manifestations included arthritis/arthralgia, lymphadenopathy and hearing loss. Hepatic cirrhosis and epilepsy were seen in one patient. None of them had bone pain or family histories. Serum monoclonal IgM gammopathy was found in both patients. MyD88 gene mutation L258P was identified in one patient. They were treated with tocilizumab and tripterygium wilfordii Hook F (TwHF) respectively and both showed good response.Conclusions: The rarity and diversity of SchS makes it difficult to be recognized. The patient in our study was the first SchS with concomitant liver and neural damage. Anti-IL-6 agents and TwHF may be alternative therapies when anti-IL-1 therapy is unresponsive or unavailable.

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Background Refractory gout is a common problem faced by experienced rheumatologists, relevant research on the difficulties of gout treatment and the understanding of "refractory gout" by rheumatologists is lacking. Methods A total of 910 Chinese rheumatologists attending the annual academic conference on rheumatism completed the electronic questionnaires. The differences in the judgment of refractory gout were analyzed between the senior experience (SE) group and the general experience (GE) group. Results A total of 751 (82.5%) respondents with relevant continuing medical education (CME) were included in our study, including 175 (23.3%) SE rheumatologists and 576 (76.7%) GE rheumatologists. Most of the rheumatologists (715, 95.2%) considered renal function insufficiency as a common complication making gout challenging to treat, while rheumatologists with different experiences had different attitudes towards obesity. For the chronic gouty arthritis patients with one or more tophi, 476 (63.9%) Chinese rheumatologists preferred febuxostat as the first-line treatment option, while 582 (78.3%) chose the maximum daily dose of 80mg for febuxostat, with a higher proportion in SE group than GE group. Meanwhile, the proportion of doctors who chose the dosage of all three uric-lowering drugs consistently with the guidelines was higher in the SE group. Moreover, 123 (70.3%) people in the SE group thought that regardless of the use of drugs for prevention of attack during ULT, the recurrence of symptoms was symptomatic of refractory gout, while fewer in GE group agreed. Similarly, 119 (68.0%) in the SE group thought that failure to achieve the targeted serum uric acid level within 6 months of standardized urate-lowering therapy could be assigned to refractory gout, while fewer in GE group agreed. In terms of personal and social factors, the SE group had a higher percentage of physicians concerned about poor compliance leading to irregular treatment and drug abuse. Conclusions Most of the rheumatologists selected uric-lowering drugs and the maximum dosage of medicines consistently with guidelines. Uncontrolled symptoms, unattainable therapeutic targets, and unimproved lifestyle and compliance of patients are difficulties that Chinese rheumatologists have to face with during gout treatment.