A Clinical Study Of 313 Patients With Primary Sj(?)gren’s Syndrome Associated Neurological Diseases

BackgroundPrimary Sjogren’s syndrome(PSS)related neurological lesions are one of the most common extragglandular manifestations of PSS.If the diagnosis and treatment of PSS patients with neurological lesions are not timely,the survival rate and prognosis of the patients may be greatly reduced.At present,there is not only a lack of consensus on the neuropathy related to primary Sjogren’s syndrome,nor a unified standard for diagnosis,and the pathogenesis of neuropathy is not clear yet,which still needs further study.Objective1.To explore the possible risk factors by retrospective analysis of the clinical manifestations and examination characteristics of primary Sjogren’s syndrome related neurological lesions.2.To explore the clinical electrophysiological and pathological features of primary Sjogren’s syndrome associated peripheral nervous system lesions(pSS-PNS).Patients and Methods1.Retrospective Case-control study.A total of 313 patients with PSS were enrolled from January 2015 to October 2020 at one hospital and met the diagnostic SS as revised by the American College of Rheumatology in 2016,twenty-two patients with pSS-PNS,45 patients with pSS-CNS and 246 patients with PSS without neurological impairment were treated as control group.2.Pathological study of nerve biopsy.The Sural nerves of patients with pSS-PNS were collected from January 1st,2020 to December 31st,2020.The specimens were fixed in 10%formalin solution,paraffin embedded,conventional section,hematoxylin and eosin staining,at last,the pathological sections which have been made were observed under light microscope.Results1.Comparison between Case Group and control group1.1 PSS-PNS was the case group,and compared with the control group,the results showed that numbness of limbs,weakness of limbs,twitching of limbs and damage of blood system were statistically significant(P<0.05).There was statistical significance in platelet count,anti-SSB antibody,anti-RNP antibody,anti-cell liner protein antibody(a-FIgG)and total protein(P<0.05).1.2 PSS-CNS was the case group,and compared with the control group,the results showed that dizziness and headache,limb numbness,limb fatigue,limb twitching,blood system damage and fever had statistical significance(P<0.05).There was statistical significance in neutrophil count,globulin quantity,albumin/globulin ratio,total albumin,immunoglobulin IgG,immunoglobulin IgM and total complement(P<0.05).2.Multivariate Logistic regression analysis2.1 PSS-PNS group:Variables independently associated with the occurrence of pSS-PNS include serum platelets,RNP-C and α-FIgG.ROC analysis of serum platelets,RNP-C and α-FIgG,and their correlation to pSS-PNS reveals substantial prediction of pSS-PNS.ROC analysis for the total risk of the combination of serum platelets,RNP-C and α-FIgG had a larger AUC(95%CI)of 0.878(0.878(0.802-0.955)(p<0.0001)),indicating an excellent prediction of pSS-PNS occurrence.2.2 PSS-CNS group:According to the results of the binary logistic regression,variables independently associated with the occurrence of pss-CNS include serum platelets,IgG,IgM and CH50.ROC analysis of serum platelets,IgG,IgM and CH50,and their correlation to pSS-CNS reveals substantial prediction of pSS-CNS.ROC analysis for the total risk of the combination of serum platelets,I gG,IgM and CH50 had a larger AUC(95%CI)of 0.790(0.642-0.887,(p<0.0001),indicating an excellent prediction of pSS-CNS occurrence.3.Electromyography and neuropathological biopsy in patients with pSS-PNSThere was no obvious rule of nerve lesion in patients with PSS complicated with peripheral neuropathy change,and the neuropathological biopsy showed vasculitis,destruction and reduction of neurons,possibly related to the destruction of macrophages.ConclusionThe clinical manifestations of primary Sjogren’s syndrome are varied,but neurological involvement may be the first symptom or even the only clinical manifestation of PSS.α-FIgG,PLT and RNP-C were the independent risk factors of pSS-PNS.The higher the level of thess three factors,the greater the possibility of peripheral neuropathy.Platelets,IgG,IgM and CH50 were the independent risk factors of pSS-CNS.The higher the level of the platelets,the lower the level of IgG,IgM and CH50,the greater the possibility of central neuropathies.There was no obvious rule of nerve lesion in patients with PSS complicated with peripheral neuropathy change,and the neuropathological biopsy showed vasculitis,destruction and reduction of neurons,possibly related to the destruction of macrophages.