• Journal of Medicine and Life Science
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• v.21 no.1
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• pp.15-19
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• 2024

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels of the body. The two most common ANCAs are myeloperoxidase ANCA and proteinase 3 ANCA. Neurological manifestations are frequent in patients with AAV, including peripheral neuropathy, meningitis, and stroke. AAV-associated ischemic stroke usually affects small vessels supplying the white matter or brainstem. This case report details the presentation and treatment course of a 70-year-old man with rapidly progressive multiple intracranial large artery involvement attributed to myeloperoxidase ANCA-associated vasculitis. Despite treatment with high-dose steroids and a rituximab infusion, the patient developed new speech difficulties and respiratory distress, and brain imaging confirmed new stroke lesions with progressive multiple intracranial large cerebral artery involvement. The patient died from SARS-CoV-2 infection 4 months after the diagnosis. This case emphasized the rare presentation of rapidly progressive large vessel involvement in a patient with myeloperoxidase ANCA-associated vasculitis despite active immunotherapy.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.89-94
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• 1992

A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.13.5-14
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• 1981

Wegener's granulomatosis is characterized by 3 criteria: 1. Necrotizing granulomas with vasculitis of upper and lower respiratory tracts 2. A systemic vasculitis 3. Focal necrotizing glomerulitis. This disease is one of the nonhealing disease in the otolaryngologic and ophthalmologic fields. A 48years old Korean male patient was seen with the complaints of nasal discharge, foul odor and frequent nasal bleeding. The patient was admitted after biopsy of the nasal cavity which diagnosed tuberculous granuloma, for biopsy of the maxillary sinus. After biopsy by Caldwell-Luc's approach this patient was complained with severe headache, visual impairment and cough. And so this patient was readmitted for further evaluation. Generally, the diagnosis was made after autopsy sometimes several years later after reevaluation of the case. Tuberculous granuloma was the pathological diagnosis on the basis of resected material in various cases. The correct diagnosis was made at autopsy occasionally. It is our intention to present this case with. literature review.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.132-137
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• 2012

Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.

• Tuberculosis and Respiratory Diseases
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• v.84 no.4
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• pp.255-262
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• 2021

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

• Investigative Magnetic Resonance Imaging
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• v.25 no.1
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• pp.53-58
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• 2021

Polyarteritis nodosa (PAN) is a systemic vasculitis involving small- and medium-sized arteries, which presents with necrotizing inflammation. PAN occurs as a systemic disease or as a limited form confined to a single organ. Few cases have been reported with single organ involvement, and even fewer have been reported with skeletal muscle involvement. Herein, we report the ultrasonography and magnetic resonance imaging findings in a rare case of PAN with limited muscle involvement in a 66-year-old man.

• Journal of Yeungnam Medical Science
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• v.30 no.1
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• pp.17-20
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• 2013

Wegener's granulomatosis is a very rare systemic vasculitis characterized by necrotizing granulomatosis. The detection of antineutrophil cytoplasm antibody (ANCA) is a valuable finding in diagnosing Wegener's granulomatosis because ANCA is positive in approximately 90 percent of patients with active, generalized Wegener's granulomatosis. But ANCA is not necessarily positive to make a diagnosis. A 59-year-old man was transferred to our hospital. He was diagnosed with lung abscess and treated with antibiotics at previous hospital. Initially, the ANCA was negative in immunofluorescence assay but we suspected Wegener's granulomatosis because of systemic inflammatory symptoms. Clinical symptoms deteriorated rapidly so we did bronchoscopic biopsy early. Wegener's granulomatosis was diagnosed according to pathologic finding that reported necrotizing granulomatous inflammation associated with vasculitis. Thus we treated with steroid then clinical symptoms and laboratory findings were improved.

• Journal of Yeungnam Medical Science
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• v.26 no.2
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• pp.130-136
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• 2009

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects the medium-sized muscular arteries, with occasional involvement of the small muscular arteries. As with other vasculitides, PAN can affect any organ system, including the cardiovascular, gastrointestinal and central nervous systems. The prognosis for patients with untreated PAN is relatively poor, with five-year survival rates of approximately 13 percent. The outcome has improved with proper therapy to approximately 80 percent survival at five years. We report here on a case of a 46 year old man with polyarteritis nodosa and who suffered from pulmonary tuberculosis.

• Korean Journal of Veterinary Research
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• v.34 no.3
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• pp.569-581
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• 1994

This study was carried out to investigate the pathogenesis of canine herpesvirus(CHV) infection in dogs. The 17 puppies, one day old, delivered from CHV seronegative 3 dams were divided into two groups. The 13 puppies were inoculated intranasally with 1ml of CHV-KK inoculum($5{\times}10^{5.6}TCID_{50}/ml$) and 4 puppies were served as control. And then the puppies were sacrificed at 2, 4, 6 and 7 days after the treatment, and collected blood, nasal mucosa, trigeminal nerve, trigeminal ganglion, bone marrow, eye, brain and other major organs. These organs were examined histopathologically and electron microscopically. The platelets of puppies infected with CHV were dramatically decreased because of the damages of vascular endothelial cells. Histopathologically, necrotizing vasculitis and neuritis were proceeded the generalized focal necrosis of all organs. Necrotic changes in trigeminal ganglion, trigeminal nerve and ventroposteriomedial nucleus of thalamus were observed in 4 puppies infected with CHV. Herpesviral particles, various forms of maturation, were observed in endothelial cells of the alveolar capillary and hepatic sinusoid with electron microscopy. These results suggest that the generalized focal necrcsis of all organs including brain and eyes in canine herpesvirus infection were resulted from generalized vasculitis, and also the hemonecrotizing meningoencephalitis was related to the necrosis of trigeminal nerve pathway.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.207-209
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• 2003

Kawasaki disease is an acute febrile vasculitis affecting primarily infants and young children. In addition to the cardiovascular involvement, it may cause inflammatory changes in various organs and body systems : digestive, respiratory, urinary, nervous and musculoskeletal. A case is reported of atypical Kawasaki disease associated with acute renal failure and necrotizing myositis in the right gastrocnemius in a 10-year-old boy. In older children, uncommon age of onset and additional features less commonly associated with Kawasaki disease may contribute to a delayed diagnosis.