BACKGROUND

Spontaneous isolated superior mesenteric artery dissection (SISMAD) is a rare vascular condition where the superior mesenteric artery is affected by dissection without involving other arteries. Its incidence is estimated at 0.06% to 0.08% globally. The possible causes include polyarteritis nodosa (PAN), an autoimmune disease affecting medium-sized arteries. SISMAD can manifest with various clinical presentations, from asymptomatic to acute bowel ischemia, warranting consideration when common causes of acute abdomen are ruled out.

This is the case of a 44-year-old female Filipino, hypertensive, who complained of abdominal pain, initially managed as intestinal amoebiasis. Abdominal examination showed a soft, non-tender abdomen with no guarding, making the symptoms disproportionate to physical examination. Due to persistence of symptoms despite a full antibiotic course, further workup was done. Computed tomography (CT) scan of the whole abdomen with contrast revealed an isolated dissection of the proximal superior mesenteric artery (SMA) with thrombosis which was confirmed on CT angiography. The diagnosis of PAN was established based on the correlation of clinical presentation, laboratory findings and imaging results. Conservative management was done and to address the thrombosis, anticoagulation with heparin was initiated. The patient was also given methylprednisolone pulse therapy and cyclophosphamide with good response. Resolution of symptoms noted and she was eventually discharged improved.

SISMAD and PAN are independently rare conditions. This unique case involved both diseases in a 44-year-old Filipino female, and to date, there have been no reported similar cases worldwide. Early diagnosis of the disease requires a high degree of suspicion and pattern recognition. This is crucial for timely treatment and improved prognosis. Furthermore, close surveillance is important to identify potential relapses even after symptom resolution.

Spontaneous isolated superior mesenteric artery dissection (SISMAD) is a rare vascular condition where the superior mesenteric artery is affected by dissection without involving other arteries. Its incidence is estimated at 0.06% to 0.08% globally. Possible causes include trauma, congenital connective tissue disorders, arteriosclerosis, and vasculitis such as polyarteritis nodosa (PAN). SISMAD can manifest with various clinical presentations, from asymptomatic to acute bowel ischemia, warranting consideration when common causes of acute abdomen are ruled out.

This is a case of a 44-year-old female Filipino, who complained of abdominal pain, initially managed as intestinal amoebiasis. Abdominal examination showed a soft, non-tender abdomen with no guarding, making the symptoms disproportionate to physical examination. Due to persistence of symptoms despite full antibiotic course, further work up was done. Computed tomography (CT) scan of the whole abdomen with contrast revealed an isolated dissection of the proximal superior mesenteric artery (SMA) with thrombosis which was confirmed on CT angiography. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), cytoplasmic antineutrophilic cytoplasmic antibodies (ANCA) and perinuclear ANCA were both negative; antinuclear antibody (ANA), C3 and C4 levels were also unremarkable. The diagnosis of PAN was established based on the correlation of clinical presentation, laboratory findings, and imaging results. She was given methylprednisolone pulse therapy and cyclophosphamide with good response. Anticoagulation with heparin was initiated to address the thrombosis. The patient had complete bowel rest, pain management, intravenous hydration, and parenteral nutrition. Resolution of symptoms noted and was eventually discharged improved.

SISMAD and PAN are independently rare conditions. This unique case involved both diseases in a 44-year-old Filipino female. Early diagnosis of the disease requires a high degree of suspicion and pattern recognition. This is crucial for timely treatment and improved prognosis. Furthermore, close surveillance is important to identify potential relapses even after symptom resolution.

A 57-year-old woman presented to vascular surgery clinic with visceral artery aneurysms that were incidentally detected during regular check-up. Imaging studies revealed occlusion of the celiac axis and severe stenosis of the superior mesenteric artery and 3 aneurysms along the posterior and inferior pancreaticoduodenal arteries, as well as the right gastroepiploic artery. Endovascular embolization of all aneurysms was rejected because of the risk of hepatic ischemia. These complicated lesion caused by polyarteritis nodosa were successfully treated using a hybrid operation with coil embolization, aneurysm resection, and antegrade aorto-celiac-superior mesentery artery bypass.
Aneurysm ; Arterial Occlusive Diseases ; Arteries ; Constriction, Pathologic ; Embolization, Therapeutic ; Female ; Gastroepiploic Artery ; Humans ; Ischemia ; Mesenteric Artery, Superior ; Mesentery ; Middle Aged ; Polyarteritis Nodosa

Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of the medium and small-sized arteries. The condition is limited to the skin and there is a lack of visceral involvement. Treatment with systemic glucocorticoids alone or in combination with azathioprine, methotrexate or cyclophosphamide, depending on the disease severity, has been shown to be effective. This paper reports the clinical case of a 53-year-old female patient with CPAN refractory to treatment with high dose glucocorticoid, methotrexate, azathioprine, and cyclophosphamide, who was treated successfully with anti-tumor necrosis factor-α therapy (adalimumab).
Adalimumab* ; Arteries ; Azathioprine ; Cyclophosphamide ; Female ; Glucocorticoids ; Humans ; Methotrexate ; Middle Aged ; Necrosis ; Polyarteritis Nodosa* ; Skin ; Tumor Necrosis Factor-alpha ; Vasculitis

No abstract available.
Peripheral Nervous System Diseases ; Polyarteritis Nodosa ; Vasculitis

Childhood polyarteritis nodosa (PAN) is a rare systemic vasculitis and the delayed diagnosis and treatment will cause high incidence of sequelae and high mortality. This article reports a girl with childhood PAN due to posterior reversible encephalopathy syndrome (PRES). The girl aged ten years was admitted to the hospital due to hypertension and convulsions for one month. She had complaints of headache, vomiting, and blurred vision before convulsions. At six months before admission, a mass was observed in the neck. The physical examination showed that she had hypertension and no abnormal findings in the central nervous system. The brain magnetic resonance imaging manifested long T1 and T2 signals, high signal intensities on fluid-attenuated inversion recovery (FLAIR) images, and iso-signal intensity on diffusion-weighted imaging in the white matter of the left occipital lobe. Therefore, the cause of convulsions was considered as PRES. Mass biopsy suggested PAN and no findings supported tuberculosis. The right kidney atrophy was observed by ultrasound examination. Emission computed tomography of the kidney showed multiple vascular stenosis and no blood perfusion in the right kidney, so PAN was confirmed. These findings suggest that PAN should be considered in patients with vasculitis who had involvements of multiple systems after excluding common vasculitis, such as Kawasaki disease and Henoch-Schönlein purpura. Biopsy and angiography should be performed as early as possible for timely diagnosis and treatment.
Child ; Female ; Humans ; Hypertension ; etiology ; Polyarteritis Nodosa ; diagnosis ; Seizures ; etiology

Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature.
Aneurysm ; Arteries ; Diagnosis ; Edema* ; Hemorrhage ; Humans ; Infarction ; Ischemia ; Lower Extremity* ; Muscle, Skeletal ; Musculoskeletal Pain ; Polyarteritis Nodosa* ; Rupture ; Systemic Vasculitis ; Thrombosis

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium- or small-sized arteries. Its diagnosis may be delayed because it is a rare disease, and patients presenting with PAN demonstrate variable clinical manifestations and non-specific laboratory abnormalities. Gastrointestinal involvement occurs in 14~65% of patients with PAN and is a significant cause of morbidity and mortality. Thus, early diagnosis is very important in PAN with gastrointestinal involvement. We report two cases of rapidly progressive PAN presenting with abdominal pain, having failed conservative treatment.
Abdominal Pain* ; Arteries ; Diagnosis ; Early Diagnosis ; Humans ; Mesenteric Artery, Superior* ; Mortality ; Polyarteritis Nodosa* ; Rare Diseases ; Vasculitis

BACKGROUND/AIMS: Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. METHODS: Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. RESULTS: Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. CONCLUSIONS: Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.
Abdominal Pain ; Biopsy ; Delayed Diagnosis ; Diagnosis ; Duodenum ; Endoscopy ; Gastrointestinal Tract ; Humans ; Immunoglobulin A ; Medical Records ; Microscopic Polyangiitis ; Polyarteritis Nodosa ; Strikes, Employee ; Takayasu Arteritis ; Ulcer ; Upper Gastrointestinal Tract ; Vasculitis*

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.
Angiography ; Arteritis ; Connective Tissue ; Ehlers-Danlos Syndrome* ; Fibromuscular Dysplasia ; Flank Pain ; Hand Joints ; Joints ; Korea ; Marfan Syndrome ; Neurofibromatoses ; Polyarteritis Nodosa ; Renal Artery* ; Skin ; Tuberculosis