The coexistence of membranous glomerulonephritis and its cause in the same biopsy: the two faces of IgG4-related kidney disease

IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by fibroinflammatory tumor-like masses that show the peculiar morphological features of storiform fibrosis, lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells and obliterans phlebitis. The disease affects virtually any organ or apparatus and is often associated with increased serum IgG4 levels. Many previously described conditions (e.g. autoimmune pancreatitis, Mikulicz’s syndrome, Küttner’s tumor, and Riedel’s thyroiditis) are now classified to be part of IgG4-RD with the characteristic clinic, serologic and pathologic features. The kidney represents an important target-organ of the disease, mainly as tubulointerstitial nephritis (TIN). Nevertheless, some cases of glomerular disease, especially membranous glomerulonephritis (MNG), have been described in IgG4-related TIN. We report a case of IgG4-related kidney disease in which the two pathological patterns, TIN and MNG, were observed simultaneously in the same biopsy.

Eponymous syndromes

This chapter discusses eponymous syndromes, including Alice in Wonderland syndrome, Arnold–Chiari malformation, Baker’s cyst, Barrett’s oesophagus, Bazin’s disease, Behçet’s disease, Berger’s disease, Bickerstaff’s brainstem encephalitis, Brown-Séquard syndrome, Brugada syndrome, Budd–Chiari syndrome, Buerger’s disease, Caplan’s syndrome, Charcot–Marie–Tooth syndrome, Churg–Strauss syndrome, Creutzfeldt–Jakob disease (CJD), Crigler–Najjar syndrome, Devic’s syndrome, Dressler’s syndrome, Dubin–Johnson syndrome, Dupuytren’s contracture, Ekbom’s syndrome, Fabry disease, Fanconi anaemia, Felty’s syndrome, Fitz-Hugh–Curtis syndrome, Foster Kennedy syndrome, Friedreich’s ataxia, Froin’s syndrome, Gardner’s syndrome, Gélineau’s syndrome, Gerstmann’s syndrome, Gilbert’s syndrome, Gilles de la Tourette syndrome, Goodpasture’s disease, Guillain–Barré syndrome, Henoch–Schönlein purpura (HSP), Horner’s syndrome, Huntington’s disease, Jervell and Lange-Nielsen syndrome, Kaposi’s sarcoma (KS), Klippel–Trénaunay syndrome, Korsakoff’s syndrome, Langerhans cell histiocytosis, Leriche’s syndrome, Löffler’s eosinophilic endocarditis, Löffler’s syndrome, Lown–Ganong–Levine syndrome, McArdle’s glycogen storage disease (type V), Mallory–Weiss tear, Marchiafava–Bignami syndrome, Marchiafava–Micheli syndrome, Marfan’s syndrome, Meckel’s diverticulum, Meigs’ syndrome, Ménétrier’s disease, Meyer–Betz syndrome, Mikulicz’s syndrome, Milroy disease, Münchausen’s syndrome, Ogilvie’s syndrome, Ortner’s cardiovocal syndrome, Osler–Weber–Rendu syndrome, Paget’s disease of the breast (PDB), Pancoast’s syndrome, Parinaud’s syndrome, Paroxysmal nocturnal haemoglobinuria, Peutz–Jeghers’ syndrome, Peyronie’s disease, Pott’s syndrome, Prinzmetal (variant) angina, Raynaud’s syndrome, Refsum disease, Romano–Ward syndrome, Rotor syndrome, Sister Mary Joseph nodule, Sjögren’s syndrome, Stevens–Johnson syndrome, Sturge–Weber syndrome (SWS), Takayasu’s arteritis, Tietze’s syndrome, Todd’s palsy, Vincent’s angina, Von Hippel–Lindau syndrome, Von Willebrand’s disease (VWD), Wallenberg’s lateral medullary syndrome, Waterhouse–Friderichsen’s (WhF) syndrome, Weber’s syndrome, Wegener’s granulomatosis, Wernicke’s encephalopathy, Whipple’s disease, Zellweger syndrome, Zollinger–Ellison syndrome.