Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a primary systemic vasculitis that affects the small vessels, and ANCA is involved as the common pathogenesis. Environmental factors such as infectious agents have been considered to play a role in triggering the autoimmunity. We report here on a case of ANCA-associated vasculitis that developed after scrub typhus. A 64-year-old male was admitted because of fever, chills, pain, weakness and hypoesthesia of his calves. He was diagnosed as having scrub typhus based on the findings of an eschar and the positive serum anti-orientia antibody. The fever continued despite the antibiotic treatment. Neurologic symptoms such as numbness, hypoesthesia and weakness began to develop in the hands, feet and calves with a persisting fever. The nerve conduction velocity study revealed mononeuritis multiplex of the superficial peroneal nerve and the median nerve. Microscopic hematuria then additionally developed, and the serology showed a positive myeloperoxidase (MPO) test. A nerve biopsy was conducted on the left superficial peroneal nerve and the result showed non-infectious systemic vasculitis of the medium-small arteries. He was diagnosed as having microscopic polyangiitis along with ANCA associated vasculitis. The fever resolved and the neurologic symptoms began to improve after steroid pulse treatment (methylprednisolone 1 g/day). The neuropathy gradually improved after discharge. We presume that the ANCA-associated vasculitis was triggered by scrub typhus.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Arteries ; Autoimmunity ; Biopsy ; Chills ; Cytoplasm ; Fever ; Foot ; Hand ; Hematuria ; Humans ; Hypesthesia ; Male ; Median Nerve ; Microscopic Polyangiitis ; Middle Aged ; Mononeuropathies ; Neural Conduction ; Neurologic Manifestations ; Peroneal Nerve ; Peroxidase ; Scrub Typhus ; Systemic Vasculitis ; Vasculitis

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis associated with antineutrophil cytoplasmic autoantibodies. Interstitial lung disease is a less recognized manifestation of MPA and has a poor prognosis. A 61-year-old man presented with persistent cough, sputum and dyspnea. Laboratory examination revealed microscopic hematuria and renal insufficiency. Perinuclear anti-neutrophil cytoplasmic autoantibodies were positive according to serological testing. Computed tomography scans showed bibasilar reticulation and honeycombing in a peripheral distribution. Therefore, renal biopsy was performed, and MPA was diagnosed. After treating with corticosteroids and immunosuppressive agents, the patient had a complete renal response but progressive interstitial lung disease. We report a case of MPA presenting with interstitial lung disease in which the patient experienced different responses in each organ.
Adrenal Cortex Hormones ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Autoantibodies ; Autoimmune Diseases ; Biopsy ; Cough ; Cytoplasm ; Dyspnea ; Hematuria ; Humans ; Immunosuppressive Agents ; Lung Diseases, Interstitial* ; Microscopic Polyangiitis* ; Middle Aged ; Prognosis ; Renal Insufficiency ; Serologic Tests ; Sputum ; Systemic Vasculitis

Primary antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) constitute a group of small vessel vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Recently, many in vitro and in vivo studies have highlighted the role of ANCA as the main pathophysiological factor in the development of AAV. Two remarkable studies on ANCA pathogenesis were recently reported. One study examined anti-lysosomal membrane protein-2, which supports the 'shared epitope' theory. The other examined the neutrophil extracellular trap that is released by neutrophils primed by ANCA. Each disease of AAV shows a broad spectrum of the clinical features and severities, which makes it difficult to diagnose and treat them. Considerable effort has been made in the past decades to improve the treatment outcomes, reduce the incidence of relapse and avoid drug toxicity. This review describes the current understanding of AAV along with a few Korean reports.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Churg-Strauss Syndrome ; Drug Toxicity ; Glycosaminoglycans ; Incidence ; Membranes ; Microscopic Polyangiitis ; Neutrophils ; Recurrence ; Vasculitis ; Wegener Granulomatosis

Peripheral nervous system involvement is common in systemic vasculitis, occurring most frequently in the polyarteritis nodosa (PAN) group of disorders and in rheumatoid vasculitis. Within the polyarteritis nodosa group of systemic necrotizing vasculitides, three subgroups have been described: classic polyarteritis nodosa, Churg-Strauss syndrome, and an overlap syndrome. Three patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated. All cases presented clinically with mononeuropathy multiples considered typical pattern of ischemic involvement of the peripheral nerve. The causes included polyarteritis nodosa, its Churg-strauss variant, and the overlap syndrome. Pain and weakness were frequent symptoms. Nerve conduction studies were abnormal In all cases. Necrotizing vasculitis was present as pathologic findings in two cases. All patients were treated with prednisolone alone or in combination with other immunosuppressive agents or with plasmapheresis.
Churg-Strauss Syndrome ; Humans ; Immunosuppressive Agents ; Mononeuropathies* ; Neural Conduction ; Peripheral Nerves ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Plasmapheresis ; Polyarteritis Nodosa ; Prednisolone ; Rheumatoid Vasculitis ; Systemic Vasculitis ; Vasculitis*

Mononeuritis multiplex may be a manifestation of systemic vasculitis or of other illnesses such as sarcoidosis, diabetes, lymphoma, and AIDS. Anti-neutrophil cytoplasmic autoantibody(ANCA) is a serologic marker for pauci-immune crescentic glomerulonephritis and systemic necrotizing arteritis, including polyarteritis nodosa, Wegener's granulomatosis and so-called ANCA-associated vasculitis. We report a case of mononeuritis multiplex due to ANCA associated vasculitis. A 46-year-old female visited hospital because of peripheral edema, pain and dyspnea. We diagnosed neuropathy associated with vasculitis by electrophysiologic study, sural nerve biopsy and angiography. The patient was treated with prednisolone and cyclophosphamide. Her symptoms and signs improved and her ANCA test was converted from positive to negative.
Angiography ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Cyclophosphamide ; Cytoplasm* ; Dyspnea ; Edema ; Female ; Glomerulonephritis ; Humans ; Lymphoma ; Middle Aged ; Mononeuropathies* ; Polyarteritis Nodosa ; Prednisolone ; Sarcoidosis ; Sural Nerve ; Systemic Vasculitis ; Vasculitis* ; Wegener Granulomatosis

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*

Antineutrophil cytoplasmic antibodies (ANCA) are closely linked to primary systemic vasculitis, and ANCA detection has became an important diagnostic hallmark of ANCA-associated vasculitis (AAV). However, it has been reported that tuberculosis is associated with positivity for ANCA and it is difficult to differentiate clinically between tuberculosis and AAV. We report a patient with the concomitant appearance of AAV and pulmonary tuberculosis. Positivity for ANCA should be carefully interpreted as indicative of AAV, especially in countries with a high prevalence of tuberculosis.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Humans ; Peripheral Nerves ; Prevalence ; Systemic Vasculitis ; Tuberculosis ; Tuberculosis, Pulmonary

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Cytoplasm ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulocytes ; Granulomatosis with Polyangiitis ; Humans ; Medical Records ; Microscopic Polyangiitis ; Neutrophils* ; Recurrence* ; Survival Rate ; Vasculitis*