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

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*

INTRODUCTION: We present a case of Polyarteritis Nodosa along the neurocutaneous distribution of the peroneal nerve with characteristic clinical, electrophysiological and histopathological features. Prevalence was estimated to be in the range of 2.0-9.0/ million in the United States. In the Philippines, no reliable data on prevalence is available.

CLINICAL PRESENTATION: We report a case of a 34-year old male, seafarer, working aboard a bulk carrier ship, who presented right lower extremity pain associated with ascending numbness and erythematous patches on the affected area. Review of the family history of similar condition or detectable neuropathies were not found.

DIAGNOSTIC WORK-UP: Initial laboratory tests showed elevated ESR, a non-reactive Hepatitis Profile and negative LE panel. EMG was done and revealed mononeuritis involving the motor and sensory components of the right peroneal nerve. A right peroneal nerve and skin biopsy were done and histopathology showed panmural necrotizing vasculitis along documented blood vessels.

TREATMENT AND FOLLOW-UP: Treatment with corticosteroids was started. Over the months of treatment, patient was noted to have episodes of increased blood pressure. Anti-hypertensive medication was started with Nifedipine 30mg/tab once a day. Renal studies done were normal. Patient's condition improved with resolution of erythematous patches over the affected area, but symptoms of mononeuropathies persisted to some degree.

Churg-Strauss Syndrome ; pathology ; Diagnosis, Differential ; Giant Cell Arteritis ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Mucocutaneous Lymph Node Syndrome ; pathology ; Polyarteritis Nodosa ; pathology ; Purpura, Schoenlein-Henoch ; pathology ; Takayasu Arteritis ; pathology ; Thromboangiitis Obliterans ; pathology ; Vasculitis ; classification ; pathology

Though systemic vasculitidis are a group of diseases with extremely low incidence and prevalence, vessels with diverse size from aorta to capillaries are involved. It has been argued how to classify and define systemic vasculitidis, especially how to discriminate poly arteritis nodosa (PAN) and microscopic polyangiitis (MPA). Since there are lots of overlapping between them, clinical manifestations, antineuclear cytoplasmic antibody (ANCA) and angiographic findings besides pathologic findings should be considered altogether. We report a case of systemic vasculitis in which crescentic necrotizing glomerulonephritis with positive perinuclear-type ANCA occurred with intraperitoneal aneurysmal rupture simultaneously. Our case can be a typical one that shows definite overlapping between PAN and MPA.
Aneurysm* ; Antibodies, Antineutrophil Cytoplasmic ; Aorta ; Arteritis ; Capillaries ; Cytoplasm ; Glomerulonephritis ; Incidence ; Microscopic Polyangiitis ; Polyarteritis Nodosa ; Prevalence ; Rupture* ; Systemic Vasculitis*

Henoch-Schoenlein purpura, also known as anaphylactoid purpura, is characterized by palpable purpura, colicky abdominal pain, gastrointestinal hemorrhage, arthralgias, and renal involvement. Histopathologically, the condition represents a vasculitis, and in fact, it may be the most common vasculitis syndrome affecting children. The pathogenesis of Henoch-Schoenlein purpura remains poorly understood, but it is postulated that an unknown antigenic stimulus causes elevation of circulating IgA and that complement activation leads to necrotizing vasculitis. All of its clinical features are attributable to wide spread vasculits. Abdominal pain is the most common gastrointestinal symptom, but intestinal bleeding and intussusception may occur. Mesenteric vasculitis is a rare but potentially serious complication of systemic vasculitis. It is reported in association with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polyarteritis nodosa, and giant cell arteritis in adult patients. Typical features are diffuse non-specific abdominal pain progressing on occasion to gastrointestinal hemorrhage, perforation, or more rarely infarction. Fortunately intestinal infarction is a rare complications, but if present carries a high chance of mortality, and swift management of the underlying vasculitis is crucial. We describe here an unusual case of a small intestinal infarction associated with Henoch-Schoenlein purpura caused by mesenteric vasculitis.
Abdominal Pain ; Adult ; Arthralgia ; Arthritis, Rheumatoid ; Child ; Complement Activation ; Gastrointestinal Hemorrhage ; Giant Cell Arteritis ; Hemorrhage ; Humans ; Immunoglobulin A ; Infarction* ; Intussusception ; Lupus Erythematosus, Systemic ; Mortality ; Polyarteritis Nodosa ; Purpura ; Purpura, Schoenlein-Henoch* ; Systemic Vasculitis ; Vasculitis

Polyarteritis nodosa is a systemic inflammatory disease resulting from necrotizing angitis of small to medium sized arteries. It involves various organs, including the gastrointestinal tract, which is involved in about 50% of all cases. Numerous complications-including abdominal pain, vomiting, and hematemesis-have been reported, but the CT findings have not been described. We report the CT findings in a case of gastric polyarteritis nodosa, and correlate these with the histopathologic findings.
Abdominal Pain ; Arteries ; Arteritis ; Gastrointestinal Tract ; Polyarteritis Nodosa* ; Stomach* ; Vomiting

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Arteries ; Giant Cell Arteritis ; Humans ; Livedo Reticularis ; Male ; Polyarteritis Nodosa ; Skin ; Thrombosis ; Vasculitis* ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis

Takayasu's arteritis is a chronic, granulomatous, large-vessel arteriopathy of unknown cause. A 45-year-old women developed multiple subcutaneous nodules preceded by fever, chills, weakness and weight loss. There were several developed pyoderma gangrenosum-like ulcerations which resulted from the breakdown of subcutaneous nodules on both forearms. A skin biopsy specimen from a forearm nodule revealed characteristics of polyarteritis nodosa including necrosis of the muscular arteries of the deep dermis and subcutaneous tissues destruction of the elastic lamella, thrombus formation, and an acute and chronic inflammatory cell infiltrates. Seven years later, the diagnosis of Takayasu's arteritis was suggested by the absence of the left radial pulse and confirmed by angiography.
Angiography ; Arteries ; Biopsy ; Chills ; Dermis ; Diagnosis ; Female ; Fever ; Forearm ; Humans ; Middle Aged ; Necrosis ; Polyarteritis Nodosa* ; Pyoderma ; Pyoderma Gangrenosum ; Skin ; Subcutaneous Tissue ; Takayasu Arteritis* ; Thrombosis ; Ulcer ; Weight Loss

Polyarteritis nodosa(PAN) is a multisystem disease characterized by necrotizing vasculitis of small andmedium-sized arteries, and in 50% of all cases there is gastrointestinal involvement. We describe a patient withPAN involving the gastrointestinal tract. A small bowel series showed nodular fold thickening, submucosal fillingdefects, shallow ulcerations, segmental luminal narrowing, and decreased peristalsis at the duodenum, jejunum, andileum; superior mesenteric arteriography showed hypervascularity and microaneurysm. Segmental resection of thesmall bowel indicated the presence of PAN.
Angiography ; Arteries ; Arteritis ; Duodenum ; Gastrointestinal Tract ; Humans ; Jejunum ; Mesenteric Artery, Superior* ; Peristalsis ; Phenobarbital ; Polyarteritis Nodosa* ; Ulcer ; Vasculitis