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.     

Successful treatment of pediatric systemic polyarteritis nodosa with cholestatic hepatitis

Polyarteritis nodosa (PAN) is a rare vasculitis in children. We report a case of 5-year-old boy with systemic PAN and cholestatic hepatitis. He had fever, abdominal pain, and gastrointestinal bleeding. Angiography revealed multiple microaneurysms in the renal, hepatic, and superior mesenteric arteries. Clinical manifestations improved slowly after immunosuppressive therapy, but liver enzyme and bilirubin levels elevated gradually. Liver biopsy findings revealed marked centrizonal canalicular cholestasis, bile duct damage, and intact hepatocyte, but there was no evidence of viral hepatitis or vasculitis. Levels of liver enzymes and bilirubin improved after two cycles of cyclophosphamide therapy. We thought that the possible etiology of elevated liver enzyme and bilirubin levels might be a manifestation of PAN.     

Polyarteritis nodosa (PAN) associated with familial Mediterranean fever (FMF): An unusual case presentation and a review of the literature

IntroductionPolyarteritis Nodosa (PAN) is a rare systemic necrotizing vasculitis and the variety in clinical presentations lead to difficulty in diagnosis. The co-incidence of PAN disease and familial Mediterranean fever (FMF) has been reported and as they share similar clinical symptoms such as fever, abdominal pain, arthralgia, and skin rash the diagnosis could be challenging.Case reportThis report is presented as one of the unusual cases of PAN with intra-parenchymal renal aneurysm rupture, which was misdiagnosed as peri-renal abscess in a patient with FMF.Case presentationA 24 year–old Syrian man presented to the Emergency Department of Tishreen Hospital with fever (39 °C), anorexia, general weakness, severe musculoskeletal pain, weight loss and flank tenderness. He had a history of FMF since he was 7 years old and used to be treated with colchicine. Laboratory investigations revealed leukocytosis, anaemia, increased erythrocyte sedimentation rate (99 mm/1st h) and C-reactive protein (CRP) (96 mg/l). Renal function, urine analysis, creatinine clearance, electrolytes and transaminases were normal. Urine and blood culture were negative. The anti-neutrophil cytoplasmic antibody, rheumatoid factor and hepatitis markers were negative. On abdominal ultrasonography, renal parenchyma was hyper-echogenic with surrounding liquid. Antibiotics were provided with no improvement. The patient developed secondary hypertension and MRI brain revealed ischemic cortical and subcortical parietal lesions. PAN was diagnosed based on renal CT angiography showing multiple micro-aneurysms. Consequently, methylprednisolone pulses and cyclophosphamide were introduced with dramatic improvement.ConclusionIncreased awareness of such clinical association of PAN and FMF and early management may improve the disease outcome.     

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?

BACKGROUND: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. OBJECTIVES: To determine the special characteristics and the prognosis of PAN in FMF patients. METHODS: A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed. RESULTS: The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis. CONCLUSIONS: When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.     

Remission of Angiographically Confirmed Minocycline-induced Renal Polyarteritis Nodosa: A Case Report and Literature Review

A 23-year-old man presented with severe hypertension. Based on his history of minocycline treatment for over three years and clinical symptoms, such as myalgias and renovascular hypertension with multiple intrarenal aneurysms, he was diagnosed with minocycline-induced renal polyarteritis nodosa (PAN). After minocycline treatment cessation and management of the hypertension, his blood pressure, renin-aldosterone levels, and urinary protein levels gradually improved. Seven and a half years later, repeated angiography found that the aneurysms had resolved. This is the first report in English describing a case of minocycline-induced renal PAN that was reversed functionally and morphologically without steroids or immunosuppressive drugs.     

Ruptured arterial aneurysm of the kidney in a patient with microscopic polyangiitis

We present the case of a 55-year-old man with ruptured arterial aneurysm accompanied by microscopic polyangiitis in the kidney. He was admitted to our hospital because of general fatigue, fever and serious numbness of his extremities. Microscopic polyangiitis (MPA) was diagnosed on the basis of cardinal symptoms, including rapidly progressive glomerular nephritis, peripheral nerve disorder and the lung abnormality, as well as positive MPO-ANCA findings. Hemodialysis had to be started on admission because of renal failure. Renal biopsy demonstrated necrotizing glomeruli, crescent formation with interstitial infiltrates. There were no immune deposits on immunofluorescence study or electron micrographs. The pathological diagnosis was necrotizing glomerulonephritis involving small and medium-sized arteries. He was treated with intravenous semi-pulse methylprednisolone therapy because of the intensely pathological renal activation and the abnormal shadow on chest X-ray. The inflammatory reaction subsequently improved, MPO-ANCA decreased and the lung lesions diminished. He complained of sharp pain of sudden onset on his left side. His hemoglobin dropped from 9.8 g/dl to 6.0 g/dl developed in the subsequent hours, but there were no sign of hemorrhage. Abdominal CT scan showed a large left-sided perinephric, intracapsular hematoma. Selective arterial angiography showed multiple aneurysms in renal and hepatic arteries. No active bleeding was present and he recovered with transfusion, supportive therapy and monitoring alone. Multiple aneurysms detected by angiography in the renal and hepatic arteries showed improvement. He is currently stable on regular hemodialysis treatment with a low dose of oral prednisolone.     

Death Resulting from Fournier Gangrene Secondary to Thrombosis of Very Large Hemorrhoids: Report of a Case

A 53-year-old male was admitted with a two-day history of abdominal pain, anal bleeding, fever, diarrhea, vomiting, and mental confusion. A diagnosis of thrombosis of very large hemorrhoids (Grade 4) was made. On the day of admission, he underwent an exploring laparotomy followed by abdominoperineal resection. The peritoneal cavity was filled with pus and blood clots. Because rectal necrosis was involved, sigmoid colostomy was imperative. Twenty-eight hours after surgery, the patient demonstrated signs of soft-tissue perineal necrosis associated with progressive pain and fever. He developed a rapidly progressive gangrene of the lower limbs and scrotum followed by acute renal and respiratory failure, and he died of sepsis. At autopsy, the cadaver showed jaundice and a large gangrene of the perineum and lower limbs. The internal organs showed features secondary to sepsis complications. To the best of our knowledge, this is the first autopsy study of a patient who died because of complications of hemorrhoids.     

Intestinal ischamemia in a child due to polyarteritis nodosa: a case report.

Polyarteritis nodosa (PAN) is a rare type of childhood vasculitis. We report the case of a 10-year-old boy who was referred to our hospital following an appendicectomy with fever, abdominal pain, vomiting, diarrhoea, weight loss, joint pains and skin rash. Shortly after admission, he developed peritonitis, and an emergency laparotomy revealed ischaemia of the jejunum which required partial resection. Histologic examination of the resected bowel showed features consistent with PAN. He was treated with steroids and cyclophosphamide. Nineteen months later, he is asymptomatic and thriving. PAN should be included in the differential diagnosis if a child with constitutional symptoms also has gastrointestinal complaints or develops an acute abdomen.     

Polyarteritis nodosa in association with subarachnoid hemorrhage

We report a 65-year-old man with classic polyarteritis nodosa (PAN) who developed subarachnoid hemorrhage. Polyarteritis nodosa was strongly suspected, however, the biopsy specimens of kidney and sural nerve showed no findings of vasculitis and the serum titer of antimyeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was negative. Cranial magnetic resonance angiography showed no findings of aneurysms. He developed subarachnoid hemorrhage (SAH) during the course and died. Autopsy confirmed fibrinoid necrosis in the medium-sized artery of multiple organs. To our knowledge, this is the first report of a case of classic PAN accompanied by SAH in which MPO-ANCA proved negative.     

A case of classical polyarteritis nodosa complicated by ulcerative colitis

Classical polyarteritis nodosa (PAN) is a term that includes patients with necrotizing inflammation of medium-sized arteries and excludes those with microscopic vessel involvement. Although gastrointestinal manifestations are not unusual in patients with classical PAN, the association with ulcerative colitis has been reported only rarely. We describe a patient with classical PAN complicated by bilateral renal artery aneurysms with subsequent rapture and perirenal hemorrhages. He was successfully treated, and the bilateral renal aneurysms resolved with steroid therapy. Two years later, the patient presented with hematochezia. Colonoscopy revealed inflamed rectal mucosa with bleeding ulcers. Histologic findings of biopsy specimens showed severe mucosal inflammation and crypt abscess. The patient was diagnosed with ulcerative colitis, and the symptoms attenuated after meselazine therapy.     

Spontaneous renal laceration as the presenting feature of polyarteritis nodosa in a patient with familial Mediterranean fever after hepatitis A infection

We report a life-threatening spontaneous renal laceration with no history of bleeding diathesis or any trauma in a patient with FMF after acute hepatitis A virus (HAV) infection. Right nephrectomy was inevitable and histological investigation of the removed right kidney revealed a polyarterits nodosa (PAN). This case underlines the possibility that simultaneous PAN and immunsupressive treatment besides colchicine should be considered for patients with FMF. Also, patients with FMF who are not immune may be vaccinated for HAV which could be a predisposing mechanism for vasculitic hemorrhage.     

Polyarteritis nodosa with mesenteric aneurysms demonstrated by angiography: report of a case and successful treatment of the patient with prednisolone and cyclophosphamide

Polyarteritis nodosa is a necrotizing angitis that predominantly affects small and medium-sized arteries. The prognosis of untreated polyarteritis nodosa is very poor. Since symptoms are diverse and no serologic test is specific for polyarteritis nodosa, the diagnosis is difficult and often delayed. We describe a patient with polyarteritis nodosa who had gastrointestinal involvement with multiple aneurysms of the inferior mesenteric artery; only abdominal angiography provided a conclusive diagnosis. Alleviation of symptoms and regression of aneurysms were observed after combination therapy of an immunosuppressive agent, cyclophosphamide, and prednisolone. We emphasize the importance of early diagnosis by angiography and aggressive therapy in patients in whom physical signs indicating definite polyarteritis nodosa are not present. Received: January 18, 1999 / Accepted: May 28, 1999     

Hepatitis B-related polyarteritis nodosa complicated by pulmonary hemorrhage

A 34-year-old man presented with fever, weight loss, paresthesia, abdominal pain, and hypertension. He had hepatitis B antigenemia, with negative antineutrophil cytoplasmic antibody, antinuclear antibody, and antiglomerular basement membrane serology results. Renal arteriography showed multiple intrarenal microaneurysms. In spite of therapy with antiviral agents (lamivudine, famciclovir), prednisone, cyclophosphamide, and plasmapheresis, renal function deteriorated. He later developed rapidly progressive dyspnea and hemoptysis. Diffuse alveolar hemorrhage was confirmed by bronchoscopy. He died of respiratory failure. The cause of pulmonary hemorrhage in this case of polyarteritis nodosa is unclear, but may include underlying capillaritis, cocaine-induced pulmonary hemorrhage, or recurrent attacks of malignant hypertension.     

Gastrointestinal bleeding and intestinal perforation due to polyarteritis nodosa in a patient with celiac disease. A case report

Complications of celiac disease could present with intestinal perforation and rarely, gastrointestinal bleeding, which are usually secondary to nongranulomatous ulcerative jejunoileitis or T-cell lymphoma. We describe the case of a 66-year-old male patient with an 8-year history of celiac disease (CD) who presented with recurrent abdominal pain and gastrointestinal bleeding. Several tests were performed to find out possible complications associated to CD. Due to an overt gastrointestinal bleeding, an arteriography was performed and signs of polyarteritis nodosa were found. Vascular disease was aggressive, and despite multiple medical and surgical treatments the patient died. As arteriography is not usually performed for the study of the complications of CD, it is possible that the association between CD and PAN has been underdiagnosed.     

Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch–Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch–Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch–Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.     

Chronicle of a death foretold: a case of catastrophic vascular Behcet’s disease

A 20-year-old man with Behcet's disease characterized by recurrent arterial aneurysms presented with a new aortic root aneurysm. This patient previously had aneurysms of the coronary arteries and vein, as well as ruptured renal artery aneurysm. Chronic maintenance immunosuppressive therapy was recommended due to the catastrophic nature of the disease, which the patient refused to take. The patient died shortly after admission. This case demonstrates the unique catastrophic natural history of vascular Behcet's disease with recurrent life-threatening arterial events, and this case stresses the therapeutic dilemma of maintenance immunosuppressive therapy in selected patients.     

Embolization of cardiac myxomas masquerading as polyarteritis nodosa.

Cardiac myxomas are rare tumors which can mimic other diseases. We describe a 19-year-old man who presented clinically with polyarteritis nodosa (PAN). Muscle biopsy showed vasculitis, but corticosteroid and immunosuppressive therapy was initially effective. Cardiac myxomas were found by echocardiogram when the disease relapsed and were resected surgically. Segmental arterial aneurysms and stenoses were found on angiography after leg ischemia. We suggest that echocardiography should be performed in all cases of clinically suspected PAN.     

Classic Polyarteritis Nodosa Presenting Rare Clinical Manifestations in a Patient with Hemophilia A

A 35-year-old patient with hemophilia A presented with rapidly progressive polyarteritis nodosa (PAN). He had been infected with hepatitis B virus (HBV) by repeated transfusion and was positive for hepatitis B surface antigen but negative for hepatitis B surface antibody. The patient presented symptoms of acute epididymitis followed by emergency admission because of acute appendicitis. On day 7 of admission, he complained of severe back pain, and computerized tomography (CT) showed massive perirenal hematoma. On day 49, mild monoplegia in the left arm suddenly developed, and CT and magnetic resonance imaging revealed multiple cerebral infarctions. Factor VIII replacement therapy was attenuated; however, cerebral infarction was progressive and extended throughout the cerebral hemispheres. He was diagnosed with classic polyarteritis nodosa (cPAN), and pulse methylprednisolone was continued. The patient died of supratentorial herniation, and autopsy revealed that vasculitis associated with intimal thickening was present in the liver, pancreas, intestine, kidneys, and larger-sized cerebral arteries. The development of cPAN appeared to have originated from chronic HBV infection, and this is the first report of cPAN in hemophilia patients. Concomitant hemorrhagic and thrombotic manifestations of cPAN are hardly treatable in patients with coagulation disorders, and the current case may represent a rare transfusion-related complication in hemophilia patients.     

An unusual presentation of periarteritis nodosa

Periarteritis nodosa is characterized by generalized inflammation of medium and small arteries that leads to thrombosis or aneurysmal dilatation. We report the case of a 30-year-old man with no preceding signs or symptoms who presented initially with a chief complaint of mild abdominal pain. He deteriorated clinically during the next six to eight hours, and developed shock secondary to a ruptured aneurysm of the left gastric artery. His postoperative course was complicated by recurrent bleeding and death within 48 hours. Our case represents a protean clinical manifestation of periarteritis nodosa and expands the differential diagnosis of acute abdominal pain.     

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis with intrarenal aneurysms and renal arteriovenous fistulaes

A 78-year-old Japanese man was admitted with a complaint of slight fever and weight loss. At admission, he tested positive for myeloperoxidase (MPO)-ANCA and had renal failure. An abdominal angiography revealed atrophy of the right kidney, two or more arteriovenous fistulae (AVF) in the right renal interlobular arteries, and multiple aneurysms in both kidneys. A renal biopsy specimen showed diffuse crescentic glomerulonephritis accompanied by tubulo-interstitial changes. This case suggests the possibility of some relationship between ANCA-associated vasculitides and the formation of aneurysms and AVFs.