genitourinary disorders
Fibrillary and Immunotactoid Glomerulopathies
Introduction
Fibrillary and immunotactoid glomerulopathies are rare conditions defined pathologically by organized deposition of nonamyloid microfibrillar or microtubular structures within the renal mesangium and basement membrane. Fibrillary and immunotactoid glomerulopathies are thought by some experts to be related disorders. They are found in about 0.6% of renal biopsy specimens, occur equally in men and women, and have been described in patients ≥ 10 years. Average age at diagnosis is about 45. Mechanism is unknown, although deposition of immunoglobulin, particularly IgG kappa and lambda light chains and complement (C3), suggests immune system dysfunction. Patients may have accompanying cancer, paraproteinemia, cryoglobulinemia, plasma cell dyscrasia, hepatitis C infection, or Systemic Lupus Erythematosus (SLE) , or they may have a primary renal disease without evidence of systemic disease. Immunotactoid glomerulonephritis in particular is commonly associated with All patients have proteinuria, > 60% in the nephrotic range. Microscopic hematuria is present in about 60%; Hypertension , in about 70%. Slightly > 50% have renal insufficiency at presentation. (See also Overview of Nephrotic Syndrome .) 