Polyarteritis nodosa-like necrotizing vasculitis in Fabry disease

Abstract A 78-year-old man presented to our hospital with a history of 10kg weight loss within 6 months previously, and general fatigue and fever for 2 and 1 months, respectively. On hospitalization, the patient was diagnosed with polyarteritis nodosa after multiple microaneurysms were observed in the liver, kidney, pancreas, and mesenteries. He achieved remission with the administration of 1,000mg methylprednisolone for 3 days, followed by prednisolone (55mg/day). Steroids were successfully tapered with no re-elevation in inflammation. Two months after the administration of steroids, the patient complained of acute abdominal pain, and developed severe acute pancreatitis. During treatment for pancreatitis, the patient died due to septic shock and disseminated intravascular coagulation. An autopsy revealed necrotizing vasculitis in the intrapancreatic arteries and ischemia of the downstream arterioles resulting in acute pancreatitis.

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Acute Necrotizing Vasculitis (Polyarteritis Nodosa?) Confined to the Nerve with Spontaneous Recovery

Rheumatology ◽

10.1093/rheumatology/34.7.694 ◽

1995 ◽

Vol 34 (7) ◽

pp. 694-695 ◽

Cited By ~ 6

Author(s):

V. DE LA SAYETTE ◽

M. MACRO ◽

PH. DIRAISON ◽

F. BERTRAN ◽

B. LECHEVALIER ◽

...

Keyword(s):

Polyarteritis Nodosa ◽

Spontaneous Recovery ◽

Necrotizing Vasculitis ◽

Acute Necrotizing

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Necrotizing vasculitis with a polyarteritis nodosa-like pattern and selective immunoglobulin A deficiency: case report and review of the literature

Journal of Cutaneous Pathology ◽

10.1111/j.1600-0560.2007.00911.x ◽

2008 ◽

Vol 35 (9) ◽

pp. 871-875 ◽

Cited By ~ 12

Author(s):

Sabela Paradela ◽

Felipe Sacristán ◽

Manuel Almagro ◽

Víctor G. Prieto ◽

Sara M. Kantrow ◽

...

Keyword(s):

Case Report ◽

Polyarteritis Nodosa ◽

Immunoglobulin A ◽

Necrotizing Vasculitis ◽

Review Of The Literature

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Convergence Paralysis as a Manifestation of Polyarteritis Nodosa

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100005424 ◽

2006 ◽

Vol 33 (4) ◽

pp. 423-425 ◽

Cited By ~ 1

Author(s):

Jessica Wylie ◽

Craig Campbell ◽

Janet Pope ◽

Jonathan Akikusa ◽

Ronald M. Laxer ◽

...

Keyword(s):

Polyarteritis Nodosa ◽

Peripheral Nerves ◽

Central Nervous System Involvement ◽

Necrotizing Vasculitis ◽

Testicular Pain ◽

American College Of Rheumatology ◽

Diastolic Hypertension ◽

Hepatitis B Antigen ◽

Visceral Arteries ◽

Blood Creatinine

Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis of medium-sized arteries. The American College of Rheumatology criteria for the diagnosis of PAN includes at least three of: 1. weight loss < 4 kg, 2. livedo reticularis, 3. testicular pain or tenderness, 4. myalgias, weakness or leg tenderness, 5. mono-or polyneuropathy, 6. diastolic hypertension, 7. elevated blood creatinine or urea, 8. hepatitis B antigen or antibody in the serum, 9. aneurysms or occlusions of visceral arteries or 10. granulocytes on small or medium sized artery biopsy. Common sites of involvement include skin, joints, kidneys, gastrointestinal tract and peripheral nerves. Central nervous system involvement has been reported in up to 40% of cases; the usual manifestations are encephalopathy, focal deficits and seizures. Descriptions of PAN in the pediatric literature has been reported in fewer than 250 children.

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Salmonella infection as a trigger for Polyarteritis Nodosa

European Journal of Case Reports in Internal Medicine ◽

10.12890/2020_001403 ◽

2020 ◽

Author(s):

Pedro Neves Tavares ◽

Patricia Alves ◽

Rita Lizardo Grácio ◽

Paula Gonçalves Costa ◽

Carla Falcão ◽

...

Keyword(s):

Polyarteritis Nodosa ◽

Clinical Case ◽

Scientific Literature ◽

Salmonella Typhi ◽

Classification Criteria ◽

Haemodynamic Instability ◽

Infectious Agents ◽

Necrotizing Vasculitis ◽

Salmonella Infection ◽

Testicular Pain

Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries. Most cases of polyarteritis nodosa are idiopathic but multiple infectious agents have been associated with this disease. We present a clinical case of a 72-year-old male with fever, diarrhoea and haemodynamic instability, diagnosed with a bacterial infection caused by Salmonella Typhi. One week after clinical resolution of the infection, the patient developed purpuric lesions with ulcers, pustules and necrotic areas accompanied by testicular pain and weight loss of 5 kg over the previous 15 days. A skin biopsy was performed and it revealed typical histologic signs of polyarteritis nodosa. The aetiologic association between bacteria of the genus Salmonella and polyarteritis nodosa has been previously described in the scientific literature but seldom meeting classification criteria and with histologic confirmation.

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Polyarteritis nodosa: An unusual cause of life-threatening intra-abdominal bleeding

Case Reports in Internal Medicine ◽

10.5430/crim.v6n4p23 ◽

2019 ◽

Vol 6 (4) ◽

pp. 23

Author(s):

Koen Verdonk ◽

Jeroen G.J. Jonkman ◽

Adriaan Dees

Keyword(s):

Blood Loss ◽

Polyarteritis Nodosa ◽

Necrotizing Vasculitis ◽

Rare Type ◽

Abdominal Bleeding ◽

Arterial Bleeding ◽

Causal Therapy ◽

Threatening Condition ◽

Life Threatening ◽

Endovascular Coil Embolization

Intra-abdominal arterial bleeding is a life-threatening condition, warranting immediate causal therapy. Polyarteritis nodosa is a rare type of necrotizing vasculitis that affects medium-sized arteries. We present a patient with severe arterial bleeding as the first symptom of polyarteritis nodosa. Because the bleeding took place intra-abdominal instead of gastro-intestinal, there was no macroscopic blood loss. She was successfully treated with endovascular coil embolization during angiography.

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