The efficacy of interferon-alpha in a patient with resistant familial Mediterranean fever complicated by polyarteritis nodosa

Familial Mediterranean fever (FMF) is a recurrent self-limiting polyserositis. Polyarteritis nodosa (PAN) complicating FMF is very rare. Here, we present a 17-year-old male patient with FMF who subsequently developed PAN 2 weeks after hepatitis A infection. This case was also complicated with perirenal haematoma, and right nephrectomy was performed. The clinical condition of the patient was improved after therapy with intravenous and oral corticosteroid and intravenous cyclophosphamide. However, the FMF attacks and vasculitic skin lesions again occurred while he was using colchicine plus immunosuppressive agents a few months later. Interferon-alpha therapy was administered and the attacks were resolved within 3 months. He has not experienced any other symptom during the follow-up period of 28 months.     

Acute hepatitis in a patient with familial Mediterranean fever

Familial Mediterranean fever (FMF) is a hereditary syndrome characterized by recurrent episodes of fever and serositis. In this report, we describe a Japanese patient with FMF and Sjögren's syndrome, in whom acute elevations of transaminase occurred. The histological findings from the liver biopsy specimens demonstrated a nonspecific hepatitis, with liver cell necrosis and interlobular inflammatory cell invasion, without the presence of interface hepatitis or bile duct injury. This case underscores the possibility that MEFV mutations contribute to hepatic inflammation, as seen in this case, by way of an alteration of the pyrin function.     

Spontaneous bilateral perirenal hematoma as a complication of polyarteritis nodosa in a patient with human immunodeficiency virus infection

We present a 29-year-old man with polyarteritis nodosa (PAN) having human immunodeficiency virus (HIV) infection. This patient fulfilled the American College of Rheumatology (ACR) 1990 criteria for PAN, and the diagnosis was confirmed by typical arteriographic findings, including microaneurysms. Due to the rupture of microaneurysms, perirenal hematomas occurred in both kidneys. Unilateral nephrectomy was performed, and renal histology confirmed that aneurysm rupture was the etiology of the perirenal hematoma. The occurrence of renal hematomas is a usual complication of PAN. However, bilateral renal hematoma during the course of HIV-associated PAN is quite rare, and to our knowledge, this would be the second case reported in the literature. When compared with other viral agents, the association of HIV with PAN may be considered rare. However, as suggested by various reports in the literature, HIV infection should always be kept in mind while evaluating patients with PAN.     

High hepatitis B virus load in a patient with severe polyarthritis nodosa

One of the extra-hepatic manifestations of hepatitis B virus is polyarteritis nodosa (PAN). It may involve medium- and small-sized arteries in any organ. Concurrency of these two diseases may be life threatening and both should be treated. Herein, we report on a patient with severe PAN and high hepatitis B virus load. The patient was an 18-year-old boy with multiple progressive wounds in the skin, referred to our center. The preliminary evaluation showed vasculitis in the skin biopsy compatible with PAN. He was treated with low dose prednisolone and lamivudine for three years. However, his condition got worse and ulcers on his leg became life threatening. The viral load was 17,000,000 copy/mL. The wound developed superimposed resistant bacterial infection. The patient was then treated with two antiviral drugs-lamivudin 100 mg/day plus adefovir 10 mg/day-and high dose cyclophosphamide (750 mg, once a month) and prednisolone (60 mg/day for one month). After six months of treatment, viral load decreased to 100,000 copy/mL and wounds healed. We concluded that high viral load of hepatitis B virus may play an important role in the severity of PAN. We recommend combination therapy with two antiviral agents with high dose of immunosuppressive drugs until both the diseases resolve significantly.     

A case of refractory cutaneous polyarteritis nodosa in a patient with hepatitis B carrier status successfully treated with tumor necrosis factor alpha blockade

We describe a patient with refractory cutaneous polyarteritis nodosa (CPAN) with hepatitis B virus (HBV) carrier status who was successfully treated with tumor necrosis factor alpha (TNF-α) blockade, using etanercept, and we review 5 similar cases. We administered etanercept because of the occurrence of repeated flares despite aggressive therapy. C-reactive protein normalization; prednisolone dose-sparing; and absence of any adverse events, including HBV reactivation with nucleotide analogue administration, or renal dysfunction, have been achieved for 8 months. TNF-α blockade should be considered for intractable CPAN.     

各型肝炎病毒单纯及重叠感染的研究

目的 探讨病毒性肝炎患者甲～戊，庚型肝炎病毒(HAV-HEV，HGV)单纯感染及重叠感染情况。方法 采用EIA法检测病毒性肝炎患者血清抗-HAV IgM，HBV标志物、抗-HCV IgM、抗-HDV IgM、抗-HEV IgM、抗-HGV IgM。结果 共检测210例病毒性肝炎患者HAV-HEV、HGV血清标志物，20例未检出(9．5％)，190例患者检出标志物阳性(90．5％)。HBV感染率89，5％(188／210，其中有34例为既往感染，占16．2％，现症感染154例，占73．3％)；HAV感染率29．0％(61／210)，HCV、HDV感染率均为8．1％(17／210)、HEV、HGV感染率依次为10．0％(21／210)、7．1％(15／210)。各临床类型中单纯感染占61．4％(129／210)，二重感染占32．4％(68／210)，以HAV HBV、HBV HDV、HBV HEV感染模式最常见，三重感染占6．2％(13／210)，以HAV HBV HDV感染模式最常见；临床上以肝炎肝硬化、重型肝炎重叠感染常见，急性肝炎最少见。结论 病毒性肝炎中HBV感染最常见，其次为HAV感染；单纯感染、二重感染多见，三重感染少见；重叠感染发生率随病情加重而增加。     

Unilateral lymphocytic pleuritis as a manifestation of familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease affecting predominantly populations surrounding the Mediterranean basin. It is the most prevalent hereditary periodic fever syndrome characterized mainly by recurrent and short attacks of fever and serositis (pleuritis, arthritis, peritonitis). Unilateral polymorphonuclear exudative pleuritis associated with fever has been reported as the solitary manifestation of the first FMF attack, in < 10% of patients. This case study describes a 30-year-old Greek man with recurrent episodes of lymphocytic exudative pleuritis associated with fever. After a thorough workup (clinical criteria and molecular genetic testing identifying homozygosity polymorphisms of the FMF gene), the diagnosis of FMF was established. Treatment with colchicine, 2 mg/d, eliminated FMF attacks. To our knowledge, this is the first well-documented case report of a patient with FMF presenting with a lymphocytic exudative pleural effusion.     

Protracted arthritis in a Japanese patient with familial Mediterranean fever

The most common arthritic involvement in familial Mediterranean fever (FMF) is acute self- limiting monoarthritis which typically lasts for 72 h. Hip joint involvement is uncommon in FMF and can result either from a process specific to this disease or from a coexisting inflammatory joint disease. We describe a 37-year-old woman with FMF and right osteoarthritis secondary to congenital hip dislocation. Periodic fever with right coxalgia lasting for 6 months was treated using colchicine. Genetic analysis revealed homozygous mutation in the MEFV gene (L110P-E148Q/L110P-E148Q), confirming the FMF diagnosis. Although the clinical presentation and course of FMF arthritis are diverse, delineating these clinical patterns may help with early recognition and treatment to prevent destructive arthritis in FMF. Clinicians should consider the possibility of FMF development in unusual monoarthritis patients with recurrent febrile attacks.     

Multineuropathy in a patient with HBV infection, polyarteritis nodosa and celiac disease

We report on the first association of celiac disease, polyarteritis nodosa and HBV infection in a patient who developed a neuropathy. On admission his general and neurological conditions were severely compromised. Haematological test revealed HBV infection and high levels of antibodies to tissue transglutaminase, endomysium, gliadin. EMG showed sensory-motor asymmetric axonal neuropathy. A sural nerve biopsy revealed fibre loss, axonal degeneration with asymmetrical distribution and fascicular ischaemia. A duodenal biopsy was consistent with celiac disease. The patient was treated with immunosuppressive and antiviral therapy, and gluten-free diet with good result. Celiac disease can be related to a higher risk of autoimmune disorders and may have contributed to the development of multineuropathy in this patient.     

A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations

The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiography sessions. Although the patient experienced no more recurrent bleeding, he died of multiorgan dysfunction syndrome resulting from sepsis 6 weeks after admission. Polyarteritis nodosa associated with FMF may follow a grave course despite immunosuppressive therapy. Arterial embolization should be considered in the presence of bleeding aneurysms in addition to immunosuppressive therapy.     

Reflex sympathetic dystrophy arising in a patient with familial Mediterranean fever

A 14-year-old girl with familial Mediterranean fever (FMF) had had acute attacks of fever, abdominal pain, and arthritis for 4 years. Her last arthritis attack was protracted, leading to reflex sympathetic dystrophy (RSD) in her right lower extremity. Physical therapy along with sympathetic ganglion block and corticosteroid therapy was used for the treatment. To our knowledge, this is the first reported case of RSD arising in a patient with FMF. Early recognition of RSD in FMF patients is important, and physical therapy should be applied along with medical treatment. Received: 26 March 1999 / Accepted: 20 July 1999     

Skin and muscle involvement as presenting symptoms in four children with familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. It is the most frequent periodic fever syndrome. In FMF, sterile peritonitis, pleuritis and arthritis are frequently seen in addition to recurrent febrile attacks. Skin and muscle involvement is less common. Here, we report four patients presented with skin lesions or myalgia. Most striking findings in those patients are the absence of other major criteria for FMF and dominancy of skin lesions or myalgia. All four patients had MEFV gene mutations on both alleles. In patients with erysipelas-like lesions or erythema nodosum along with arthritis/arthralgia or recurrent myalgia, FMF should be kept in mind.     

Etanercept in the treatment of arthritis in a patient with familial Mediterranean fever

Familial Mediterranean fever (FMF) patients may present with different joint complaints, one being the 'protracted attack' that lasts for weeks. We present a 15 year-old boy with polyarthritis (right wrist, knee, shoulder, and both ankles) while on colchicine treatment for FMF. His polyarthritis was resistant to treatment with prednisolone and methotrexate, and etanercept was instituted (0.8 mg/kg/week). He responded dramatically to etanercept and remained in full remission, although the drug was stopped at 4 months due to social and financial causes. We suggest that anti-TNF drugs may be an alternative for resistant attacks. However the timing and dosage, as well as efficacy, need to be further studied.     

肝癌患者肝炎病毒感染血清学标志检测的分析

目的：了解肝癌患者的肝炎病毒感染状况。方法：采用酶联免疫吸附试验（EUSA）检测了肝癌、其他肿瘤及非肿瘤患者肝炎病毒感染的血清学标志。结果：在肝癌患者中乙型肝炎（HBV）HBsAg、HBeAg、抗-HBc、丙型肝炎（抗-HCV）、丁型肝炎（抗HDV）、戊型肝炎（抗-HEV）和庚型肝炎（抗-HGV）阳性率分别为28．1％、10．7％、29．8％、13．2％、5．0％、7．4％和4．5％;其他肿瘤患者为10．1％、7．1％、12．6％、4．5％、2．0％、2．8％和2．5％;非肿瘤患者为2．5％、2．0％、3．0％、2．0％、1．5％、2．5％和2．0％。肝癌患者HBV病毒与其他肝炎病毒混合感染率为14．9％。结论：肝癌与HBV病毒感染有密切关系,其抗-HEV和抗-HDV阳性率也高于非肿瘤患者,对献血员血液及患者在输血或手术前进行肝炎病毒血清学标志的检测,是预防肝炎病毒感染和区分医患责任的重要措施。     

Seroepidemiology of hepatitis A virus in Kuwait

AIM： To find the current seroepidemiology of hepatitis A virus （HAV） in Kuwait. METHODS： A total of 2851 Kuwaitis applying for new jobs were screened.RESULTS： HAV-positive cases were 28.8%; 59% were males and 41% were females. The highest prevalence was in the Ahmadi area. High prevalence was among the group of non-educated rather than educated parents. This is the first study in Kuwait demonstrating the shifting epidemiology of HAV. CONCLUSION： This study reflects the need of the Kuwaiti population for an HAV vaccine.     

Efficacy of anakinra treatment in a patient with colchicine-resistant familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. The serious complication of FMF is AA-type amyloidosis, which can result in end-stage renal disease. Although colchicine is effective in the majority of patients, there is no established treatment for those who are resistant or intolerant to colchicine. We herein report the efficacy of anakinra in a 52-year-old Turkish patient with FMF, secondary amyloidosis and renal transplant, who was resistant to colchicine treatment.