Mikroskopik poliarteritis ve ailevi akdeniz ateşi birlikteliği: Vaka sunumu
Amaç: Mikroskopik poliarteritis (MPA) ve Ailevi Akdeniz Ateşi (AAA) birlikteliği çocuklarda nadirdir. AAA tanısı ile izlenen ve MPA saptanan bir hastanın sunulması ve bu vaka ile AAA hastalarında amiloidoz dışı böbrek tutulumu ve tedavisinin tartışılması amaçlamıştır. Vaka: 4 yaşındaki vaka kusma, karın ağrısı ve ishali takiben gelişen oligüri ile başvurdu. Akut böbrek yetersizliği olan vakaya 7 gün akut periton diyalizi uygulandı. Hipokomplemantemi, hematüri, proteinüri, anemi saptanmadı. Aynı dönemde renal biyopside mezenjial proliferatif değişikliklerle birlikte segmental bazal membran kalınlaşması görüldü. Beş doz intravenöz (IV) metil prednizolon (30 mg/kg/doz) verilmesinin ardından oral kortikosteroid (1 mg/kg/gün) bir ay sürdürüldü. Hastanın idrar miktarı ve kreatinin klirensi 2 hafta içinde normale döndü. İzleminde ilk başvurusundan 2 yıl sonra 2-3 gün süren karın ağrısı ataklarının olması, atak sırasında kan fibrinojen düzeyinin yüksek bulunması ve apendektomi öyküsü olması nedeniyle vakada AAA düşünüldü ve M694V/V726A mutasyonu saptandı. Kolşisin 1.5 mg/gün başlandı ve AAA atak sıklığı azaldı. Sekiz yıl sonra hastada proteinürinin ortaya çıkması üzerine yapılan ikinci renal biyopside IgG, IgM ve C3 depolanması gösteren fokal segmental glomerüloskleroz saptandı. Otoimmun antikor incelemesinde p-ANCA pozitifti. Fokal segmental glomerülosklerozun mikroskobik poliarteritisin bulgusu olması ve p-ANCA pozitifliği nedeniyle MPA tanısı konulan hasta IV metil prednizolon ile tedavi edildi. Bu tedavi ile proteinüri kayboldu ve renal fonksiyonlar normal olarak seyretti. Sonuç: AAA ve vaskülit ilişkisi bilindiğinden AAA hastalarının multi-sistemik bulgular açısından da yakın izlemi önemlidir. Herhangi bir böbrek etkilenmesi durumunda amiloid dışı böbrek tutulumu mutlaka gözönüne alınmalıdır.
Familial mediterranean fever associated with microscopic polyarteritis: A case report
Aim: Amyloidosis is the most common renal involvement in Familial Mediterranean Fever (FMF). Microscopic Polyarteritis (MPA) associated with FMF is very rare in children. In this report, our aim was to present a patient with FMF associated with MPA, and to discuss non-amyloid renal involvement in FMF and its treatment. Case: A Four-years-old girl was admitted with oliguria following vomiting, abdominal pain and diarrhea. Peritoneal dialysis was performed for 7 days because of acute renal failure. There were no hypocomplementemia, hematuria, proteinuria and anemia at the time of admission. Segmental basement thickness with proliferative changes in mesengium was observed in renal biopsy. Due to the hitopathological changes, intravenous pulse methyl prednisolone (MP) (30 mg/kg/dose) therapy followed by oral MP (1 mg/kg/day) was administered for one month. Oliguria and creatinine clearence improved in the patient within two weeks. During follow-up, two years after the first presentation, FMF was considered because of fever and abdominal pain attacks recurring at two week intervals and continuing for 2-3 days, increased fibrinogen level in one of these attacks and an appendectomy in her history. M694V/V726A mutation was determined in genetic analysis. Colchicine 1,5 mg/day was started, and frequency of the attacks was decreased. Eight years later, when she was 15 years old, proteinuria was established and a second renal biopsy was performed. Focal segmental glomerulosclerosis (FSGS) with IgG, IgM, C3 deposition was determined. P-ANCA was found to be positive. Because FSGS was a sign of MPA and pANCA was positive, MPA was diagnosed in the patient. Intravenous MP was administered. Proteinuria disappeared and creatinine clearence normalized with this therapy. Conclusion: Due to the known relationship between FMF and vasculitis, the patients with FMF should be followed-up closely in respect of multisystemic symptoms. Non-amyloid renal lesions should be considered as well, in all FMF patients with a finding of renal involvement.
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