C1Q NEPHROPATHY: CASE REPORTS AND LITERATURE REVIEW
Autori:
Krešimir Galešić, Ivica Horvatić, Danica Batinić, Danko Milošević, Marijan Saraga, Merica Glavina Durdov, Danica Galešić Ljubanović
Sažetak
C1q-nefropatija oblik je glomerulonefritisa definiran dominantnim nalazom C1q-imunosnih depozita u glomerulima. Bolest je rijetka. Najčešće se javlja u djece i mlađih odraslih. Klinički se najčešće očituje nefrotskim sindromom premda su opisani i drugi klinički sindromi kojima se ova bolest može manifestirati. Etiologija bolesti nije poznata, a patogeneza je najvjerojatnije imunosne prirode. Bolest je često rezistentna na glukokortikoide i druge imunosupresive i tada obično uzrokuje kronično zatajenje bubrega. U ovom radu prikazano je deset bolesnika s C1q-nefropatijom koja je potvrđena karakterističnim histološkim promjenama u tkivu bubrega koje je dobiveno biopsijom. Nijedan bolesnik nije imao kliničkih ni laboratorijskih nalaza karakterističnih za sistemski eritemski lupus. U svih su bolesnika komponente komplementa C3 i C4, kao i nalaz ANA, anti-dsDNA te ANCA-protutijela bili uredni.
Summary
C1q nephropathy is considered a form of glomerulonephritis, defined by histological findings of dominant C1q immune deposits in renal biopsy. It is a rare disease, most often manifested in children and young adults. The most common clinical manifestation of the disease is nephrotic syndrome, but other renal syndromes could also be found. The cause of the disease is not known, but the immune pathogenesis could be assumed. Often, resistance to glucocorticoid or other immunosuppressive therapy is present, potentially leading to chronic renal insufficiency. We present ten patients with renal biopsy and clinical findings of C1q nephropathy. None of the patients had clinical or serological manifestations of systemic lupus. All patients had normal findings of C3 and C4 components of complement, as well as normal ANF, anti-dsDNA and ANCA antibodies.
