microscopic polyangeitis

We report a case of progressive outer retinal necrosis (PORN) in a patient of microscopic polyangitis (MPA), being treated with immunosuppressive drugs such as cyclophosphamide and rituximab. Her aqueous tap was positive for Varicella Zoster virus and she was treated with oral and intravitreal antivirals, along with discontinuation of one of the immunosuppressive agents, i.e. rituximab, which might have led to reactivation of the virus causing necrotizing retinitis lesions. Rituximab and cyclophosphamide are extremely potent drugs, which are necessary to manage immunological disorders such as MPA...

OBJECTIVE: Primary systemic vasculitis (PSV) is a rare disorder in children and difficult to distinguish from other diseases. However, appropriate diagnosis and prompt treatment will affect on the morbidity and mortality of intractable PSV. In this study, we conducted a nationwide survey in Japan, to clarify epidemiology and clinical outcome of PSV. METHODS: We had sent survey questionnaires to most of the Japanese institutions that employed pediatricians, requesting the number of patients with refractory PSV who were diagnosed and treated between 2007 and 2011...

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Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases. Although the aetiology of AAV is uncertain, it is likely that genetic and environmental factors contribute. We report the unusual case of two brothers presenting with AAV with differing clinical pictures and differing ANCA specificity. There is a recently identified difference in genetic risk factors associated with ANCA specificity, making it surprising that first-degree relatives develop AAV with differing clinical and serological features...

INTRODUCTION: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. CASE PRESENTATION: A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB...

Silica (silicon dioxide) occupational exposure has been linked to both pulmonary and extra-pulmonary toxicity. Silicosis is the major pulmonary toxicity, which has also been associated with the development of collagen-vascular disease and with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, especially perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA). The most common pulmonary manifestations of microscopic polyangitis (MPA) are interstitial fibrosis and alveolar hemorrhage. We describe a patient who had unusual presentation of microscopic polyangitis, characterized by lung hemorrhage, rapidly progressive glomerulonephritis, pleuropericarditis and pulmonary embolism that was associated with a history of silica exposure and radiologic evidence for silicosis...

AIM: The vasculitis diseases granulomatosis with polyangiitis (GPA) and microscopic polyangitis (MPA) are the two major forms of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). A recent genome-wide association study has shown that the genes HLA-DPB1 and HLA-DQ conferred susceptibility to GPA and MPA, respectively. We investigated the linkage between putative AAV-related genes (HLA-DPB1, ARHGAP18, CD226, CTLA-4, MOSPD2 and PRTN3) and AAV in a Han Chinese population...

Systemic necrotizing vasculitis may be idiopathic or associated with a variety of diseases of known etiology. A typical example is polyarteritis nodosa, which is characterized by fibrinoid necrosis and severe inflammation leading to destruction of the wall, narrowing of the lumen, and interference with blood circulation. In addition to the idiopathic form, histologically similar lesions are seen in hepatitis B, rheumatoid arthritis, Kawasaki mucocutaneous lymph node syndrome, and other diseases. Microscopic polyangitis involves mainly small vessels-venules more often than arterioles-but occasionally also small arteries...

A 76-year-old woman suffering from granulomatosis with polyangitis (GPA) developed organizing pneumonia with positive antineutrophil cytoplasmic antibodies and microscopic hematuria. Prednisolone improved the hematuria and radiological findings; however, after tapering the dose of prednisolone, a posterior left atrial wall mass was detected in association with a fever. Both regressed spontaneously, although secretory otitis media and sinusitis were noted; the resected sinusitis specimen exhibited vasculitis highly suggestive of GPA...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

OBJECTIVE: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of children with ANCA-associated vasculitis. METHOD: Fifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included. Their data of pre-diagnosis status, clinical manifestations, renal pathology, treatment and prognosis were reviewed retrospectively...

BACKGROUND: Pulmonary fibrosis is a manifestation of microscopic polyangitis (MPA), and often precedes the detection of MPA. The prevalence and sequence of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and MPA in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF) have not been precisely elucidated. METHODS: We enrolled 61 consecutive patients with IPF and measured the MPO-ANCA titers at initial presentation and during the follow-up period...

A 77-year-old gentleman, a holidaymaker in the area, presented with a 2-month history of cough productive of rust-coloured sputum and exertional dyspnoea. His initial chest x-ray showed bibasal interstitial shadowing. He then deteriorated rapidly to type 1 respiratory failure with acute renal failure. A CT chest showed bilateral alveolar haemorrhage. Testing for p-anti-neutrophil cytoplasmic antibody was strongly positive, with an anti-myeloperoxidase antibody titre of 198 units/ml (normal range 0-6 units/ml)...

To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG), seven as microscopic polyangitis (MPA) and one as Churg-Strauss syndrome (SCS). The median age of the patients was 57.7 years and the sex-ratio (M/F) was 1...

In December 2008, a 69-year-old Japanese woman was admitted to the Department of Otorhinolaryngology because of hearing impairment due to bilateral exudative otitis media, and was discharged without complete recovery despite conventional treatment. Two weeks later, she was readmitted for worsened deafness, numbness, gait disturbance, and general fatigue. She was referred to our department for general investigation. On admission, laboratory examination revealed severe inflammatory signs and active nephritic urinary sediments...

An 81-year-old man was hospitalized because of fever and pain in the temporal region. Temporal artery biopsy revealed temporal arteritis; steroid therapy was started. Chest computed tomography and kidney biopsy revealed interstitial pneumonia and necrotizing crescentic glomerulonephritis, respectively. Because his myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) level was 215.0 U/mL, a diagnosis of microscopic polyangitis (MPA) was made. The patient was discharged after reduction of the steroid dose...

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We describe the clinical case of 80 years, caucasian woman, with personal history of osteoporosis and chronic normochromic normocytic anemia (NN). She had a three month history of myalgias of the girdle, stiffness in the morning, exceeding 1 hour, associated with inflammatory arthralgia of the small joints of hands and feet. Complementary exams showed normocytic normochromic anemia with Hg 9.8 g/dL; ESR 44 mm/h; CRP 7 mg/dL. Given the profile suggestive of Polymyalgia Rheumatica started prednisolone 10 mg/day with favorable clinical response...

We encountered 2 cases of microscopic polyangitis (MPA) with preceding interstitial pneumonia. The patients were elderly men, and radiological findings showed a UIP pattern in both cases. Furthermore, ANCA-associated vasculitis had been suspected since the first admission in case 1, because MPO-ANCA tests were positive, and the surgical lung biopsy revealed vasculitis in a honeycomb lesion. A diagnosis of idiopathic pulmonary fibrosis was made in case 2 because no findings of vasculitis were pathologically confirmed, and MPO-ANCA was not examined...