Naslov
A case of polyarteritis nodosa presenting as a rapidly progressive glomerulonephritis

Autori
Čužić, Snježana ; Božić, BOrka ; Šćukanec-Špoljar, Mira

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Proceedings of "Autoimmune systemic diseases" / International editorial board(Cosyns, Ferluga, Vizjak, Ivanyi, Šćukanec-Špoljar, Čikeš, Rozman) - Ljubljana : Medicinska fakulteta, Ljubljana, 1999

Skup
XXXth Memorial meeting for Professor Janez Plečnik - Autoimmune diseases

Mjesto i datum
Ljubljana, Slovenija, 02.12.1999. - 03.12.1999

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Polyaretritis nodosa; vasculitis; glomerulonephritis; crescentic

Sažetak
A 49-year old female patient was admitted to the hospital due to recurrent swelling of small joints and knee.The patient was treated with Sulfosalazine for several months and did well for a year and a half.In wimter 1998, the patient was treated for otitis media with antibiotics.In spring 1999 she was again admitted to the hospital because of abdominal pain, nausea, swelling of joints and edema around the eyes.There were signs of acute nephritic syndrome with hematuria, hypertension and proteinuria(up to 6 gr/24 h).Furthermore, elevated levels of urea(44.6 mmol/l)and creatinine(436 mmol/l)in serum, as well as decreased creatinine clearence were found.A suspicion of rapidly progressive glomerulonephritis of unknown etiology was raised. The kidney biopsy showed a widespread glomerular sclerosis with mesangial widening of preserved glomerular areas.There was a medium muscular artery in the specimen with, in serial slides, acute and healing lesions of vasculitis.A fibrinoid necrosis of arterial wall with polymorphonuclear infiltration as well as fibrous thickening of the arterial wall and recanalization of occluded arterial lumen were observed.In the arterial wall subtle deposits of C3 were detacted.In the interstitium around affected artery numerous mononuclear cells accumulated.At sites far from affected artery, scarce mononuclear cell infiltrate was observed with foci of tubulitis.A diagnosis of polyarteritis nodosa(PAN)was made. Subsequently, angiography was performed and multiple arterial aneurysms involving branches of both renal arteries, art.mesenterica superior and art.colica sinistra were observed.There were abnormalities on the eye fundus.Further laboratory investigation revealed positive anti-DNA autoantibodies, ANCA were negative.The patient was treated with corticosteroids and cyclophosphamide.After therapy there was improvement of proteinuria and renal function. We present a rare case of PAN diagnosed upon kidney biopsy.In the series of 61 kidney biopsies from patients with vasculitis reported by Serra A. et al.only two had extraglomerular vasculitis without necrotizing glomerular lesions.In another study by Akikusa B. et al., 33% cases of renal vasculitis exhibited histological changes compatible with PAN, but all were accompanied with necrotizing glomerulitis.Here described tubulitis is one of the hallmarks of renal vasculitis, although there has not been enough attention given to it in the literature.Our patient presented with clinical signs that are in concordance with those of the majority of patients with systemic vasculitis affecting kidney, regardless whether the glomerular lesions were present or not.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA