| BackgroundIgG4-related disease(IgG4-related disease,IgG4-RD)is a general term for a complex class of rheumatic immune diseases,and its manifestations are diverse.Although the spectrum covers the various systems and organs,it manifests itself in intraspinal occupancy IgG4-related diseases are rare.Imaging examinations and other related laboratory examinations currently lack satisfactory examination indicators with sensitivity and specificity.They are difficult to diagnose in clinical work and are easily confused with other infectious diseases and lesions.ObjectivesTo improve the comprehensive understanding of IgG4-related diseases,especially the diagnosis and differential diagnosis of IgG4-related diseases that manifest as intraspinal space-occupying lesions.Materials and MethodsRepoted a patient admitted to Department of Neurosurgery,Shandong Provincial Hospital Affiliated to Shandong University,He presented with cervical spinal cord compression due to intraspinal occupying.ToAnalyze the diagnosis and treatment process,postoperative pathological examination,and supplementation of pathological resultsResultsThe patient was a 61-year-old man who was admitted to hospital in October 2016 because of "unreliable pain in the neck and bilateral shoulders associated with numbness for more than one month."The other hospital gave the patient examinations of MRI and CT,and he had received anti-infective and anti-tuberculosis treatment,no obvious effect and the condition exacerbated,after admission the patient taken the lumbar puncture and review MRI,patients Lumbar puncture showed protein abnormalities,and there was no significant change in the position of the spinal canal.After receiving the anti-infective therapy and adjusting the anti-infective drugs after admission,there was no obvious improvement in the patient's condition and finally received surgical treatment.Surgical pathology results showed that:fibrous tissue hyperplasia with chronic inflammatory cell infiltration,plasma cell infiltration is more obvious,no granuloma reaction,can not be excluded from IgG4-related diseasesIn order to clarify the diagnosis of IgG4-related diseases and improve other relevant examinations,supplemental examination of patients'hormone levels,review of postoperative MRI,and improvement of immunohistochemical staining of pathological sections,etc.,and at the same time taken more related examinations of differential diagnosis.Finally,The pathological results of the patient suggest typical plasma fibrosis with plasmatic infiltrates.Although no significant increase in IgG4,but the abnormal IgE index in this patient,while the absolute value of IgG4 immunohistochemically positive cells and IgG positive cut-off values meet the diagnostic criteria,Comprehensive literature discussion,the patient eventually diagnosed with IgG4-related disease(IgG4-related sclerosing dural meningitis)ConclusionsThis patient is an IgG4-related sclerosing dural meningitis of the spine leading to cervical spinal cord compression.There is a certain difficulty in the diagnosis due to the characteristics of IgG4-related diseases,both laboratory tests and imaging studies.It is difficult to provide an effective diagnosis basis.At the same time,the serum level of IgG4 in this patient is not high for typical level.The literature reports also suggest that the IgG4 serum level cannot be used as a reliable diagnostic condition.Currently,comprehensive diagnostic criteria and diagnostic procedures are widely used.More dependent on pathological examination results,especially further immunohistochemical examination,and there is no specific organ diagnostic criteria for IgG4-related sclerosing meningitis,which makes it difficult to make a final diagnosis.The patient once received anti-tuberculosis treatment and anti-infective therapy during the entire course of treatment,further embodying the difficulties in clinical treatment and diagnosis during the development of IgG4-related diseases,that is,the appearance of similar infections and specificities.Diseases such as infections and tumors need to be taken seriously by clinicians and discreet diagnostic treatments.SignificanceReviewing the overall treatment process,patients who have undergone empirical anti-tuberculosis and anti-infective therapy should fully integrate existing imaging data to look for evidence of IgG4 related system and organ involvement(such as serum IgG4 levels,allergies,and other immune proteins.Spectral examination results,etc.)Improving the relevant immune protein profiles,serum protein electrophoresis,and rheumatoid immune laboratory tests can assist differential diagnosis and even avoid invasive surgical intervention. |
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