melphalan and Vasculitis

We reviewed the literature in 2007 on 3 groups of systemic diseases affecting the kidney: lupic nephropathy (LN), small vessel vasculitis (SVV) and renal amyloidosis. A systematic review of 268 patients with LN pooled from 4 studies found that mycophenolic acid (MPA) in the induction phase caused more remissions and achieved greater renal survival than cyclophosphamide (CP), confirming it as a valid alternative to CP. Using a protocol including rituximab and MPA in the induction phase (14 days), MPA alone without corticoids is effective and safe in the maintenance phase. Rituximab has also been successfully used in CP-resistant forms of LN, where it reduces clinical activity and mesangial proliferation. Plasma exchanges achieve better results than bolus corticoids in SVS with severe renal failure. Complications are severe. Anti- TNF-alpha agents provide no benefit in this indication. Prolonged administration of low-dose corticoids reduces the incidence of relapses. MPA is an alternative to CP if this drug cannot be administered. Good results are achieved with rituximab in CP-resistant forms. According to a controlled trial, treatment of AL amyloidosis with dexamethasone and melphalan has equivalent results to highdose melphalan and rescue with hematopoietic stem cell transplant. In AA amyloidosis, eprosidate slows the rate of progression of renal failure.

Topics: Adrenal Cortex Hormones; Amyloidosis; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Combined Modality Therapy; Cyclophosphamide; Drug Therapy, Combination; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Kidney Diseases; Kidney Failure, Chronic; Lupus Nephritis; Melphalan; Mycophenolic Acid; Plasma Exchange; Randomized Controlled Trials as Topic; Recurrence; Rituximab; Vasculitis

Focal and segmental glomerulosclerosis (FSGS) is a common histological finding in patients with proteinuria. The natural history of the condition varies, and although it may be responsive to therapy, FSGS is an important cause of end-stage renal disease. FSGS can be caused by a variety of conditions, but it has been reported rarely in association with a plasma cell disorder.. Mayo Clinic databases were queried and cross-referenced for FSGS and plasma cell disorders. The diagnoses were confirmed, and relevant clinical and laboratory data were abstracted.. A cohort of 13 patients with "idiopathic" FSGS and a monoclonal plasma cell disorder were identified. Four patients had myeloma, and 9 patients had monoclonal gammopathy of undetermined significance. Patients treated for myeloma experienced improvement in their renal lesion, and the latter relapsed when the myeloma relapsed.. We show that FSGS and plasma cell disorders are temporally and epidemiologically linked. Therapy for the underlying plasma cell disorder can lead to resolution of FSGS. The emerging molecular pathogenesis of both FSGS and myeloma also provides potential mechanisms that link the 2 conditions together. Thus, physicians must rule out a plasma cell proliferative disorder in patients with FSGS before concluding that the renal lesion is idiopathic. Moreover, FSGS may respond favorably after the underlying plasma cell disorder is controlled.

Topics: Aged; Amyloidosis; Antibodies, Monoclonal; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Cohort Studies; Combined Modality Therapy; Comorbidity; Cytokines; Databases, Factual; Dexamethasone; Female; Glomerulosclerosis, Focal Segmental; Humans; Immunoglobulin Light Chains; Kidney; Male; Melphalan; Metabolic Clearance Rate; Middle Aged; Multiple Myeloma; Myeloma Proteins; Paraneoplastic Syndromes; Paraproteinemias; Peripheral Blood Stem Cell Transplantation; Prednisone; Prevalence; Proteinuria; Recurrence; Remission Induction; Retrospective Studies; Time Factors; Vasculitis

We present a case report of a patient with cryoglobulin-vasculitis caused by multiple myeloma in Durie/Salmon stage I refractory to conventional therapy modalities treated with high-dose chemotherapy followed by autologous blood stem cell transplantation. While clinical symptoms of vasculitis disappeared, elevated cryoglobulin levels persisted. Therefore we conclude that the relief of symptoms was caused by an immunomodulation induced by the autologous stem cell transplantation.

Topics: Antineoplastic Agents, Alkylating; Cryoglobulins; Humans; Male; Melphalan; Middle Aged; Multiple Myeloma; Peripheral Blood Stem Cell Transplantation; Transplantation, Autologous; Vasculitis

Topics: Female; Humans; Melphalan; Middle Aged; Ovarian Neoplasms; Vasculitis