Lupus nephritis is one of the most severe Systemic Lupus Erythematosus features, defining treatment modality and prognosis. Our retrospective study, including 178 patients treated for lupus nephritis during 23 years with mostly cyclophosphamide-based initial regimens followed by azathioprine or mycophenolic acid, demonstrates 84.8% of renal response with 19.2% of flares, 15-year patient survival 78.7% and kidney survival 76.3%, and low damage accrual. Both patient and kidney survival significantly differ for subgroups that achieved complete or partial renal response and nonresponders: patient 15-year survival 95% versus 65% versus 35%; kidney 15-year survival 100% versus 58% versus 0%, respectively. 51% (24 out of 47) of patients evaluated at the end of the study period sustained complete renal response; however, only 9 of them had 0 disease activity according to SELENA SLEDAI scale, while 13 patients had scores 2–4 due to the serological abnormalities only. We conclude that (1) initial treatment with cyclophosphamide followed by azathioprine is effective and can be used in agreement with International Guidelines until the evidence for biological treatments benefits becomes available; (2) complete and even partial renal response have positive prognostic value, and failure to achieve renal response negatively influences kidney and patient survival; (3) the validity of complete renal response in SLE is questioned by the absence of conventional definition of SLE remission.
Multiple myeloma is a plasma-cell dyscrasia presenting with generalized neoplastic changes in bones, accompanied by impaired haematopoiesis, susceptibility to infections, and end-organ damage. However, the clinical picture of myeloma might be quite different from the classic manifestation, patients can present with renal disease, dominating or precluding multiple myeloma features. Diagnosis and differential diagnosis demand renal tissue pathology evaluation, most often kidney pathology demonstrate cast-nephropathy, AL amyloidosis, and light-chain deposition disease. Light-chain proximal tubulopathy is less frequently reported variant of paraproteinemic kidney damage in patients mostly not demonstrating overt clinical features of multiple myeloma. This lesion is almost always induced by the excess of kappa light chains, excreted through the kidney and reabsorbed in the proximal tubule cells, which manifests with moderate CKD, and, rarely, with acquired adult Fanconi syndrome. We present two cases of myeloma with light-chain proximal tubulopathy. None of our two patients met multiple myeloma criteria at admission, and only kidney biopsy, which discovered light-chain proximal tubulopathy, prompted further work-up, in turn resulted in the diagnosis of multiple myeloma. Our cases demonstrate some peculiarities, making them rare even in the setting of light-chain proximal tubulopathy, which is rare itself. Case one is an extremely infrequent example of light chain lambda deposition in the proximal tubules, case two demonstrates acute kidney injury on the top of chronic kidney disease, unusual for the light-chain proximal tubulopathy. We conclude that kidney biopsy should be performed, unless contraindicated, in all patients with proteinuria and/ or impaired kidney function of unknown origin; and that the chemotherapy with bortezomib-based regimens is effective for treatment of myeloma with light-chain proximal tubulopathy.
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