mycophenolic-acid and Paraproteinemias

In C3 glomerulopathy, uncontrolled complement C3 activation via the alternative pathway results in glomerular C3 deposition and, in many cases, progressive renal failure. Despite advances in understanding of C3G pathogenesis over the last few years, there are no proven treatments. We describe a patient in whom C3 glomerulopathy was associated with renal impairment and elevated serum free kappa light chains. An initial response to corticosteroids was followed by relapse once steroids were weaned, prompting use of mycophenolate mofetil to maintain remission. We discuss some of the diagnostic and therapeutic issues surrounding C3G, including in the setting of monoclonal gammopathy.

Topics: Complement C3; Enzyme Inhibitors; Glomerulonephritis; Humans; Male; Middle Aged; Mycophenolic Acid; Paraproteinemias; Remission Induction

The authors retrospectively reviewed the efficacy of mycophenolate mofetil (MMF; mean dose, 2 g/day given for an average of 14 months) in 21 patients with immune demyelinating polyneuropathy. There were no significant differences in the median strength, sensory, or Rankin scores before and after treatment. In patients with immunoglobulin (Ig) M neuropathy, the M-protein decreased by 22%. Three patients with chronic inflammatory demyelinating polyneuropathy (30%) and one patient with IgM neuropathy (13%) improved. The authors found that MMF induced a modest benefit in ~20% of their patients and stabilized patient condition, allowing reduction of steroid or IVIg therapy.

Topics: Adrenal Cortex Hormones; Adult; Aged; Combined Modality Therapy; Drug Evaluation; Female; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Immunosuppressive Agents; Lymphocyte Activation; Male; Middle Aged; Mycophenolic Acid; Nausea; Neurologic Examination; Paraproteinemias; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retrospective Studies; Treatment Outcome

The term "trabecular myopathy" has been used to designate a syndrome resembling limb-girdle muscular dystrophy in which the predominant pathological feature is an abundance of lobulated or trabecular muscle fibers. However, the validity of this nosological entity has not been verified. Herein we describe a 63-year-old man with a severe, progressive myopathy who exhibited the typical pathological features of both trabecular myopathy and nemaline myopathy in association with a biclonal gammopathy. In this case, adult-onset nemaline myopathy was probably the primary disease process. The diagnostic significance of trabecular muscle fibers remains uncertain.

Topics: Biopsy; Disease Progression; Humans; Immunosuppressive Agents; Inclusion Bodies; Male; Middle Aged; Muscle Fibers, Skeletal; Muscle Weakness; Muscle, Skeletal; Mycophenolic Acid; Myopathies, Nemaline; Paraproteinemias; Prednisone