Scandinavian Journal of Rheumatology

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Familial Mdierranean Fever and Polyarteritis Nodosa S. Ozen, U. Saatci, F. Balkanci, N. Besbas, A. Bakkaloglu & T. Tacal To cite this article: S. Ozen, U. Saatci, F. Balkanci, N. Besbas, A. Bakkaloglu & T. Tacal (1992) Familial Mdierranean Fever and Polyarteritis Nodosa, Scandinavian Journal of Rheumatology, 21:6, 312-313, DOI: 10.3109/03009749209099250 To link to this article:

Published online: 12 Jul 2009.

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Date: 22 March 2016, At: 18:54


Familial Mdierranean Fever and Polyarteritis Nodosa S. Ozen, U. Saatci, F. Balkanci, N. Besbas, A. Bakkaloglu and T. Tacal Department of Pediatric Nephrology and Radiology Hacettepe University, Faculty of Medicine, Ankara, Turkey

Ozen S. Saatci U, Balkanci F, Besbas N, Bakkaloglu A , Tacal T. Familial Mediterranean Fever and Polyarteritis Nodosa. Scand J Rheumatol 1992; 21: 312-313.

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Key words: familial Mediterranean fever, polyarteritis nodosa

Familial Mediterranean Fever (FMF) is characterized by recurrent and self-limited attacks of fever and Polyserositis (1). Vasculitic diseases have been occasionally reported to accompany FMF ( 2 4 ) . In fact, it has been suggested that the occurrence of Polyarteritis Nodosa (PAN) in patients with FMF is significantly higher than in the population (2). In this report we present the youngest child with FMF and PAN, so far found. Case report A 6-year-old Turkish girl who was on colchicine treatment for her typical attacks of FMF, was admitted with complaints of myalgia and arthralgia in the extremities. She had lost weight in the recent months and complained that the abdominal pain was more severe and different. Physical examination revealed a blood pressure of 130/90 mm Hg. A macular rash in the form of livedoreticularis was noted on the lower extremities, as well. Laboratory investigations showed an erythrocyte sedimentation rate (ESR) of 120 mm/hr, and a fibrinogen level of 456 (N:200-400). Creatinine phosphokinase level was within normal limits. A skin biopsy was performed but did not show any pathological changes. Since her abdominal pain became more severe and she developed tenderness, an emergency abdominal ultrasonography was performed, showing a massive retroperitoneal hematoma. In the operation a right perinephric and renal hematoma was observed. Post-operative ultrasonographies revealed the persistence of a

Seza Ozen, Kuleli sok. 9/2, Gazi Osman Pasa, 06700 Ankara, Turkey Received 21 February 1992 Accepted 7 August 1992


right subcapsular hematoma on the dorsal side of the kidney. An angiotensin-converting-enzyme-inhibitor was started for her persistent hypertension, while colchicine treatment was continued. Since a diagnosis of PAN was suspected a digital subtraction angiography was performed: Non-selective abdominal aortography revealed two interlobar saccular aneurysms in the right and one smaller aneurysm in the left kidney (Fig. 1). Multiple microaneurysms at the more distal segments of the right interlobar arteries were demonstrated in the subsequent selective right renal angiography , in addition to those described above. The diagnosis of PAN was thus confirmed and oral corticosteroid 2 mg/kg/day was added to her treatment. She continues to be in remission six months later.

Fig. I. Non selective aortography , demonstrating bilateral aneurysms. The early arterial capillary staining of the right kidney is pale.

Familial Mediterranean Fever and PAN

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FMF has been reported in association with some vasculitis syndromes and especially PAN (2-4). The coexistence of these two diseases seem to be too frequent to be explained by chance (4). Glikson et al. have speculated that a relationship was present between streptococcal infections and the development of PAN in these patients (2). We believe it is hard to draw such a conclusion since streptococcal infections are rather endemic in the countries where FMF is fairly common. Further studies may enlighten whether some specific antigenic factors are present in FMF that dispose to PAN. Another significant feature of the coexistence of these two diseases is the high incidence of perirenal hematoma as observed in our case. Although this is a well defined but rate complication of PAN, it occurred in more than half of the patients with FMF+PAN (2-4). It is tempting to speculate that

an augmented insult to the endothelium is present in these cases. A conscientious evaluation of severe flank pain in such patients, which may well be mistaken for a FMF attack, may save the patient from the potentially fatal complication of perirenal hematoma.

References Majeed HA, Barakat M. Familial Mediterranean fever (recurrent hereditary polyserositis) in children: analysis of 88 cases. Eur J Pediatr 1989; 148: 636-41. Glikson M , Galun E, Schlesinger M et at. Polyarteritis nodosa and familial mediterranean fever: A report of 2 cases and review of the literature. J Rheumatol 1989; 16: 5369. Sachs D, Langevitz P, Morag B, Pras M. Polyarteritis nodosa and familial mediterranean fever. Br J Rheumatol 1987; 26: 139-41. Schlesinger M , Oren S, Fano M , Viskoper JR. Perirenal and renal subcapsular haematoma as presenting symptoms of polyarteritis nodosa. Pastgrad Med J 1989; 65: 681-3.


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Familial Mediterranean fever and polyarteritis nodosa.

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