• International journal of thyroidology
• /
• v.11 no.2
• /
• pp.176-181
• /
• 2018

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been reported in Graves' disease patients treated with antithyroid drugs (ATDs), especially propylthiouracil. ATD-induced ANCA-associated vasculitis usually involved the kidneys followed by the respiratory organs and skin. The treatment of ANCA-associated vasculitis induced by ATDs is to stop ATD therapy immediately, which often leads to an overall good prognosis. We report a case of ANCA-associated vasculitis in the peripheral nerves of the lower extremities in a 66-year-old woman who was treated with methimazole (MMI) for Graves' disease. To our knowledge, this is the third case of peripheral nervous system (PNS) involvement of ATD-induced vasculitis and the first case of PNS vasculitis associated with MMI.

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

• The Korean journal of internal medicine
• /
• v.39 no.3
• /
• pp.524-536
• /
• 2024

Background/Aims: This study applied the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with systemic sclerosis (SSc) and investigated the frequency of overlap syndrome of SSc and AAV (SSc-AAV-OS). Methods: Among the 232 patients diagnosed with SSc, 105 with signs suggestive of small- or medium-vessel vasculitis, which were defined as the present of interstitial lung disease (ILD), peripheral neuropathy, or suspected renal vasculitis, were included in this study and analyzed. Results: Among the 105 SSc patients, the detection rate of ANCA was 19.0%. When the 2022 ACR/EULAR criteria were applied, the frequency of SSc-AAV-OS was 20.0%, which was much higher than 1.7% reported with previous criteria for AAV. ANCA positivity contributed to the reclassification of SSc-AAV-OS more than ANCA negativity in SSc patients with signs suggestive of small- or medium-vessel vasculitis. Conclusions: The frequency of SSc-AAV-OS in SSc patients with signs suggestive of small- or medium-vessel vasculitis at diagnosis was 20.0%. Therefore, we suggest that physicians should perform ANCA tests in SSc patients exhibiting signs suggestive of small- or medium-vessel vasculitis and apply the new criteria for AAV.

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

• Childhood Kidney Diseases
• /
• v.26 no.2
• /
• pp.86-90
• /
• 2022

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis requires prompt diagnosis and treatment, since renal function at the time of diagnosis is significantly associated with renal outcomes. Here, we report two pediatric patients with ANCA-positive glomerulonephritis initially presenting with hematuria, mild proteinuria, and normal renal function. The first patient with a high myeloperoxidase-ANCA titer (>134 IU/mL) was diagnosed with rapidly progressive glomerulonephritis based on renal biopsy and treated with immunosuppressive therapy after 10 months of follow-up. The second patient with a low myeloperoxidase-ANCA titer (11 IU/mL) maintained normal kidney function without medication. Two cases showed different clinical course according to ANCA titer.

• Journal of Yeungnam Medical Science
• /
• v.30 no.1
• /
• pp.17-20
• /
• 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
• /
• v.21 no.1
• /
• pp.101-107
• /
• 2004

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.213-218
• /
• 2001

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)

• Childhood Kidney Diseases
• /
• v.17 no.1
• /
• pp.6-12
• /
• 2013

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.

• Tuberculosis and Respiratory Diseases
• /
• v.58 no.1
• /
• pp.78-82
• /
• 2005

Propylthiouracil(PUT) is a drug which used at Grave's disease. But PTU has recently been observed to associated with antineutrophil cytoplasmic antibody(ANCA)-positive vasculitis resulting in, infrequently, diffuse alveolar hemorrhage. We report the case of a patient who developed diffuse pulmonary hemorrhage after she had been taking PTU for two years. She had received a diagnosis of Grave's disease at two years ago. The serologic study was positive for ANCA with myeloperoxidase(MPO) specificity. Bronchoalveloar lavage(BAL) fluid analysis revealed hemosiderinladen macrophages. Such findings suggested propylthiouracil-induced dffuse pulmonary hemorrhage associated with antineutrophil cytoplasmic antibody. To our knowledge, this represents the first documentation in a case of PTU-induced diffuse pulmonary hemorrhage in Korea.