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Tumor Discovery                                                      Ureteral IgG4-related disease in urology



            fibrosis, and sclerosis of individual glomeruli. The renal
            tubule lumens were dilated, accompanied by dystrophic
            changes in the tubular epithelium.
              Within the lymph node tissue, slight fibrosis was
            noted with plasticization of the interfollicular spaces and
            sinuses. Morphological changes were most consistent with
            tumor-like fibroinflammatory lesions of the ureter, chronic
            interstitial nephritis, and sinus histiocytosis of the lymph
            nodes.
              For   the  differential  diagnosis  of  IgG4-RD,
            immunohistochemistry  (IHC) was  performed using
            antibodies against CD138, anaplastic lymphoma kinase   Figure 5. Immunohistochemical image showing periphlebitis. There is a
                                                               positive reaction with antibodies to CD138 in the cytoplasm of plasma
            (ALK), kappa and lambda light chains, and IgG4. IHC   cells. Magnification: ×200.
            specimens revealed a normal kappa/lambda ratio in the
            infiltrate, and no ALK expression was observed. Staining
            with antibodies against CD138 confirmed the presence of
            an admixture of plasma cells in the infiltrate (Figure 5), the
            majority of which were IgG4-positive (Figure 6).
              The CD138+/IgG4+ ratio exceeded 80%, with over 200
            IgG4-positive plasma cells observed in the field of view at
            400× magnification.
              Morphological  and  IHC  changes  corresponded  to
            IgG4-related ureteritis (so-called segmental ureteritis)
            and IgG4-related interstitial nephritis. No evidence of
            malignant tumor growth was found in the examined
            tissue. According to the morphological and IHC results,   Figure  6.  Immunohistochemical  study  showing  positive  staining  with
            the patient exhibited a serum IgG4 concentration of   IgG4 antibodies in the tumor. Magnification: ×400.
            149 mg/dL (reference range: 10 – 135 mg/dL). Thus, based
            on the clinical, morphological, and laboratory findings, we   describe in their clinical observation the presence of pain
            established the diagnosis of IgG4-RD of the left ureter. The   in the right quadrant of the abdomen , while Lei et al.
                                                                                              [16]
            patient is currently under dynamic follow-up, and no signs   reported in their observation the manifestation of IgG4-
            of disease recurrence have been observed.          related ureteral disease with pain in the left lumbar region .
                                                                                                           [12]
            3. Discussion                                      Additionally, Zhong  et al. describe the manifestations
                                                               of the disease, including lumbar and abdominal pain,
            IgG4-RD   has  recently  been  identified  as  an  fever, myalgia, and weight loss . In a comprehensive
                                                                                         [13]
            independent disease, characterized by the presence   study analyzing treatment outcomes in 65  patients with
            of  lymphoplasmacytic  infiltration  of  affected  tissues   IgG4 genitourinary diseases, Teng et al. reported that at the
            accompanied by hyperproduction of IgG4 [1-3] . This   time of the initial presentation, all patients exhibited varying
            condition is observed in various organs, exhibiting a diverse   degrees of renal failure due to impaired urodynamics and
            range of clinical manifestations, occasionally mimicking   damage to the tubular apparatus of the kidneys . In our
                                                                                                     [10]
            malignant neoplasms [7,8] . While IgG4-related nephritis   observation of IgG4-RD of the ureter, the patient’s clinical
            and retroperitoneal fibrosis have been described in detail   manifestations included left lumbar pain and anorexia,
            in the literature, instances of IgG4-related ureteral diseases   which were also non-specific. Laboratory tests revealed
            have been documented in only 15 clinical cases [12,13,15] . The   an increase in blood creatinine to 131 µmol/L and urea to
            management of patients with ureteral IgG4-RD remains   7.1 mmol/L, corresponding to the initial stage of chronic
            challenging, given the complexities associated with both   kidney disease.
            diagnosis and treatment.                             The complexity of the diagnosis is further compounded
              The complexity of the diagnosis largely stems from   by the presence of radiological signs typically associated
            the subtle clinical course of the disease and the absence   with ureteral tumor lesions. Lei  et al. provide a
            of specific clinical manifestations. Notably, Williams et al.   radiographic description of an IgG4-related ureteral lesion


            Volume 2 Issue 3 (2023)                         4                          https://doi.org/10.36922/td.1766
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