SECONDARY GN (GENERAL) Flashcards
What is caused by circulating antibodies directed against an antigenic site on type IV collagen in the GBM?
ANTI-GBM DISEASE
What is is a systemic vasculitic syndrome with involvement of the skin, gastrointestinal tract, and joints in association with a characteristic glomerulonephritis?
HSP
What is a is a rare giant cell arteritis of unknown pathogenesis characterized by inflammation and stenosis of medium-sized and large arteries, with a predilection for the aortic arch and its branches?
TAKAYASU ARTERITIS
What is a systemic vasculitis with giant cell involvement characterized by segmental transmural inflammation of medium-sized and large elastic arteries by a mixed infiltrate of lymphocytes, monocytes, polymorphonuclear leukocytes?
TEMPORAL ARTERITIS
What is a systemic necrotizing vasculitis primarily affecting medium-sized, muscular arteries, often at branch points, producing lesions of varying ages with focal aneurysm formation?
PAN
What is now considered an autoimmune “conformeropathy” involving perturbation of the quaternary structure of the α345 NC1 hexamer of type 4 collagen?
GOODPASTURE SYNDROME
What disease is characterized by a chronic inflammatory cell infiltration of the exocrine salivary and lacrimal glands and is associated with the “sicca complex” of xerostomia and xerophthalmia?
SJOGREN SYNDROME
What disease is characterized by the extracellular deposition of characteristic fibrils in various organs?
AMYLOIDOSIS
What disease should be suspected in all patients with circulating serum monoclonal M proteins?
AMYLOIDOSIS
What are the 3 types of Fibrillary renal disease?
- FIBRILLARY
- IMMUNOTACTOID
- FIBRONECTIN GLOMERULOPATHY
What is a systemic disease caused by the overproduction and extracellular deposition of a fragment of monoclonal immunoglobulins?
MIDD
What is the disease condition wherein patients have an abnormal circulating monoclonal IgM protein in association with a B-cell lymphoproliferative hematologic disorder?
WALDENSTROM MACROGLOBINEMIA
What is an inherited (X-linked) disorder of collagen α3α4α5(IV) with almost inevitable progression to ESKD, and is frequently associated with hearing loss and ocular abnormalities?
ALPORT SYNDROME
What is the other name of Nail-Patella Syndrome?
HEREDITARY OSTEO-ONYCHODYSPLASIA
What is an autosomal dominant condition affecting tissues of both ectodermal and mesodermal origin, manifested as symmetrical nail, skeletal, ocular, and renal anomalies?
Nail–patella syndrome (NPS)
What disease that results from an X-linked inborn error of glycosphingolipid metabolism involving a lysosomal enzyme, α-galactosidase A (also known as ceramide trihexosidase), and caused by mutations of the GLA gene.
FABRY DISEASE
What is the defect in Fabry Disease?
What is the mutation in Fabry Disease?
GLA GENE
What is a disorder that is associated with insulin resistance in which there is loss of fat, which may be localized to the upper part of the body or more diffuse?
LIPODYSTROPHY
What is a familial disorder characterized by proteinuria, anemia, hyperlipidemia, and corneal opacity?
LCAT
What disease is a characterized by dysbetalipoproteinemia and lipid deposition in the kidney, leading to glomerulosclerosis and renal failure?
Lipoprotein glomerulopathy (LPG)
Refers to the pattern of sclerosing glomerulopathy in HIV-infected patients
HIVAN
