1 Departments of Infectious Diseases, Nephrology and
2 Rheumatology, Cerrahpaa Medical School, University of Istanbul, Istanbul, Turkey
Correspondence to:
M. Basaranoglu, Maresal Cakmak Mah., Mehtap Sok., Gül apt. No. 6/8, Box: 34 600, Güngören, Istanbul, Turkey.
SIR, The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established [1, 2]. Although perirenal haematoma has been reported more frequently in patients with FMF associated with PAN [1, 2] compared to PAN alone [3], subcapsular splenic haemorrhage has not been described previously in FMF co-existing with PAN. Here we report a case of FMF complicated by spontaneous perirenal and subcapsular splenic haemorrhage secondary to PAN.
A 44-yr-old male was admitted to hospital in April 1997 with a history of malaise, high fever, arthralgia, severe myalgia for 2 weeks and severe abdominal pain for 2 days following an episode of acute pharyngitis. He described recurrent attacks of self-limited abdominal pain and fever for 27 yr. Ten years ago, during one of these attacks, he was diagnosed as having appendicitis and appendectomy was performed. Although he originated from an area where FMF is prevalent, he denied a positive family history. Physical examination revealed widespread palpable purpura over the abdomen, back and lower extremities, especially prominent over the thighs and buttocks. There was also periorbital and palmar oedema, arthritis of the left ankle and s.c. nodules. He had a temperature of 39°C, blood pressure of 180/100 mmHg and a heart rate of 112/min. Neurological examination and funduscopy were normal.
Laboratory evaluations then revealed haemoglobin of 12 g/dl, white blood cell count of 10 800/mm3 , with 89% neutrophils and 11% lymphocytes, and platelet count of 311 000/mm3 . The erythrocyte sedimentation rate (ESR) was 135 mm/h, C-reactive protein (CRP) 247 mg/l (normal: <5 mg/l), ferritin 876 ng/ml (normal: 20495 ng/ml for male) and antistreptolysin O titre 180 U/l (normal: 50200 U/l). Blood urea nitrogen (BUN) was 65 mg/dl and creatinine 2.7 mg/dl. Urinalysis revealed proteinuria (2 g/24 h) and microscopic haematuria (25 red blood cells/mm3 ). Muscle enzymes were within normal limits. HBsAg, anti-HBctotal, anti-HCV and anti-HIV were negative. The following were either negative or within normal limits: cryoglobulin, antineutrophil cytoplasmic antibodies, antinuclear antibody, anti-double-stranded DNA antibody, rheumatoid factor, C3 and C4. Biopsy of the skin lesions showed leucocytoclastic vasculitis, while immunofluorescence examination showed IgA and IgM deposits. A repeated rectal biopsy was negative for amyloid deposition.
He developed massive gastrointestinal haemorrhage and acute flank pain, first on the right then on the left side on the fourth and seventh days of his admission. Endoscopic examination of the upper and lower gastrointestinal tract did not reveal any pathology. Bilateral perirenal haematoma was diagnosed by abdominal ultrasonography and computed tomography (CT) (Fig. 1). Renal arteriography revealed multiple microaneurysms suggesting PAN. A major pseudoaneurysm in the left kidney was embolized. He was put on an i.v. cyclophosphamide (1 g/month) together with methylprednisolone 60 mg oral/day and colchicine 2 mg oral/day regimen. Twenty-seven days after initiation of the treatment, he developed another attack of severe pain located over the left hypochondrium. Subcapsular splenic haemorrhage was detected by CT and splenectomy was performed. Histological examination showed aneurysm formation and necrotizing panarteritis of medium-sized arteries.
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Although Turkey belongs to a part of the world where FMF is prevalent, the mean duration between the onset and the diagnosis of the disease is still 7 yr [4]. In this case, there was a striking delay of 27 yr before the diagnosis was finally made. He fulfils the recently described criteria for FMF by Livneh et al. [5]. Our diagnosis of FMF was based mainly on the history of recurrent, self-limited, short-lived attacks of fever and abdominal pain, which persisted after appendectomy, and response to colchicine therapy. In September 1997, a missense mutation of the MEFV gene on the short arm of chromosome 16 was described [6], but the diagnosis of FMF will remain primarily clinical until the sensitivity and specificity of the genetic testing are confirmed.
The prevalence of PAN is 6/100 000 in the general population and the incidence is 0.7/100 000 [7]; however, its prevalence in FMF is 1% [2]. PAN associated with FMF has been observed in a significantly younger age group (mean 20.8 yr) compared to PAN alone (mean 45 yr) [1]. Although the role of hypersensitivity, streptococcal infection, genetic and/or autoimmune mechanisms has been speculated to explain the increased frequency of PAN in FMF, there is no satisfactory explanation for the association of these entities, which share many common symptoms and signs like fever, abdominal pain, haematuria, skin involvement, occult blood in stool and arthritis.
Although splenomegaly has been observed in FMF with [8] or without amyloidosis [4], spontaneous splenic rupture has not been described before. However, this complication has been reported rarely in isolated PAN [9].
To our knowledge, the present case is the first patient described with FMF and co-existing PAN who developed spontaneous subcapsular splenic haematoma along with bilateral perirenal haematoma. We should, therefore, be aware of this complication in patients with FMF who develop acute flank pain, because such patients deserve immunosuppressive treatment besides colchicine.
This work was presented in spoken presentation form as `A case of Familial Mediterranean Fever and polyarteritis nodosa complicated with perirenal and subcapsular splenic haemorrhage' at the 1st International Conference on Familial Mediterranean Fever (FMF), Jerusalem, Israel, 1997.
References