microscopic polyangeitis

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...

No abstract text is available yet for this article.

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...

A 74-year-old woman developed fever, numbness of legs and glomerulonephritis. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) were positive in her serum, and she presented with acute renal failure. She was also simultaneously diagnosed as having both gastric and duodenal cancers. Complete resection of both cancers and renal biopsy was performed. Some glomeruli showed cellular crescentic changes, while submucosal necrotizing vasculitis of small vessels was noted adjacent to the gastric cancer...

We report a rare case of bilateral hearing loss and diffuse alveolar hemorrhage associated with microscopic polyangitis (MPA). A 75-year-old woman complained of hearing loss. Two months later, she was admitted due to rapidly progressing dyspnea. Chest radiography and CT scan showed diffuse bilateral consolidations. Mechanical ventilation was required for respiratory insufficiency. Laboratory data demonstrated anemia and renal failure. Steroid pulse therapy and antibiotics were initiated. On day 2, bronchoalveolar lavage showed fresh blood-like fluid, which suggested diffuse alveolar hemorrhage (DAH)...

Vasculitis affecting the peripheral nerves predominantly manifests as subacute, progressive, asymmetric sensorimotor polyneuropathy or mononeuritis multiplex, and more rarely as painful mononeuropathy, pure sensory neuropathy, neuropathy of the cranial nerves, plexopathy, or as autonomic neuropathy. Vasculitic neuropathy may occur isolated or non-isolated (systemic) together with involvement of other organs. Systemic vasculitis with involvement of the peripheral nerves is further subdivided into primary (Takayasu syndrome, giant cell arteritis, classical panarteritis nodosa, thrombangitis obliterans, Kawasaki disease, Churg-Strauss syndrome, Wegener granulomatosis, cryoglobulinemic vasculitis, Behcet disease, microscopic polyangitis, Schoenlein Henoch purpura) or secondary systemic vasculitis (autoimmune connective tissue diseases, vasculitis from infection, sarcoidosis, malignancy, drugs, radiation, or diabetes)...

Besides the classic "renal crisis", a well known form of acute renal failure sometimes complicating scleroderma, another type of acute renal injury, even rarer and not well recognized, does exist: a crescentic glomerulonephritis associated with ANCA, and more seldom with anti-GBM antibodies, which is often (but not always) secondary to the use of D-penicillamine. We report the case of a 70 years-old female who presented with a severe acute renal failure accompanied by positive anti-MPO ANCA as well as anti-GBM antibodies...

Thrombotic thrombocytopenic purpura (TTP) is in rare cases associated with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, and often has a fatal outcome. We report the case of a 77-year-old woman with microscopic polyangitis (MPA) presenting with TTP. Rapidly progressive renal dysfunction and paralysis and sensory disturbance of the left lower limb were noted. Serum creatinine was 3.95 mg/dl, and the titer of myeloperoxidase-ANCA was 238 EU. She was diagnosed with MPA, and high-dose methylprednisolone was initiated, followed by 60 mg/day of prednisolone...

Mesangioproliferative glomerulonephritis (MesPGN) consists 10% of the total renal biopsy of glomerulonephritis. Aim of the present study was to find out clinicopathological changes in MesPGN and differences between diffuse and focal variety. MesPGN was seen mostly in young adults with mean age of 28.63 years for males and 26.3 years for females. Male predominance was noted (M:F ratio - 1.4:1). About 70.83% patient presented with edema feet, followed by hypertension (29.19%), fever (16.66%), oliguria, nausea and vomiting (10...

Following an international consensus conference held in Chapel Hill, North Carolina, USA, vasculitides have been divided based on the size of the vessel involved in the inflammatory process. Thus, the terms large, medium and small vessel vasculitis emerged. Wegener's granulomatosis, microscopic polyangitis and Churg-Strauss syndrome are described as small vessel vasculitides. Pulmonary involvement is a characteristic feature of both Wegener's granulomatosis and Churg-Strauss syndrome, less so in microscopic polyangitis...

Systemic vasculitides are characterized by different histological aspects: fibrinoid necrosis of the arterial wall, giant cell arteritis, non-necrotizing arteritides without granuloma or giant cell infiltration. Each histological form is associated with a spectrum of diseases with variable clinical expression: giant cell angeitides, such as Takayasu's arteritis and giant cell arteritis, necrotizing angeitides, such as polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis, Henoch-Schönlein purpura or Churg-Strauss syndrome...

No abstract text is available yet for this article.

Vasculitis is characterized by an inflammatory reaction of vessel walls with damage to the dependent tissues. Forms of vasculitis which frequently have skin changes include leukocytoclastic angiitis (LcV), Henoch-Schönlein purpura (HSP), cutaneous polyarteriitis nodosum (cPAN), erythema elevatum et diutinum (EED) and urticarial vasculitis (UV). In other forms of vasculitis, systemic manifestations predominate but there are a variety of skin changes. Kawasaki disease (MK), cryoglobulinemic vasculitis (kV), Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangitis (MPA) belong to this group...

OBJECTIVE: To study the clinicopathologic features of microscopic polyangitis (MPA), and to compare the differences in anti-neutrophil cytoplasmic autoantibody (ANCA)-positive and ANCA-negative patients, as well as in ANCA-positive cases with or without glomerular immunoglobulin deposition. METHODS: Thirty-four biopsy-proven cases of MPA were retrieved from the archival files of the Department during the past 7 years. The clinicopathologic characteristics between ANCA-positive and negative patients, as well as between ANCA-positive cases with and without glomerular immunoglobulin deposition, were compared...