Case Of Microscopic Polyangiitis Research Articles

ANCA Associated vasculitis- A case of microscopic polyangiitis with proliferative glomerulonephritis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a collection of diseases, characterised by destruction and inflammation of small and medium vessels. Microscopic Polyangiitis (MPA) is part of an ANCA-associated vasculitis (AAV).The clinical signs diverge and disturba number of organs such as the kidneys, lungs, stomach and intestine. Skin manifestations such as purpuric, urticarial, nodular, ulcerative, livedoid and necrotic skin lesions were common as in other vaso-occlusive disorder. Morphology and added features aid the diagnostic approach. Here, we report a diagnostically challenging case of microscopic polyangiitis with progressive glomerulonephritis.

A case of microscopic polyangiitis initially presented with erythema multiforme‐like skin eruptions

A case of microscopic polyangiitis initially presented with erythema multiforme‐like skin eruptions

A case of microscopic polyangiitis with periaortitis treated with rituximab

A case of microscopic polyangiitis with periaortitis treated with rituximab

A case of microscopic polyangiitis in an elderly patient presenting predominantly with cholecystitis successfully treated with mizoribine

A case of microscopic polyangiitis in an elderly patient presenting predominantly with cholecystitis successfully treated with mizoribine

A case of microscopic polyangiitis with skin manifestations in a seven-year-old girl

A case of a 7-year-old girl with microscopic polyangiitis (MPA) with a skin eruption characterized by maculopapular, erythematous and purpuric lesions on the face, elbows, and knees is presented. Anti-neutrophil cytoplasmic autoantibodies (ANCA) with myeloperoxidase specificity (MPO-ANCA) were identified. Chest X-ray and computed tomography scan revealed diffuse infiltrates in both lung fields, suggesting alveolar hemorrhage. Microscopic hematuria was detected but a renal biopsy showed no abnormalities. Histological examination of a skin biopsy from a purpuric papule showed leukocytoclastic vasculitis of the small vessels in the entire dermis. The patient was treated with prednisolone and mizoribine, resulting in an improvement in the skin lesions except for those on the knee.

Microscopic polyangiitis presented with polyneuropathy of lower extremities and ANCA-associated glomerulonephritis: Case Report

We present the case of a 67-year-old female patient with microscopic polyangiitis presented with polyneuropathy of lower extremities and rapidly progressive glomerulonephritis. Disease had started as a pain and weakening of muscular strength first in the left and than in the right leg. Electromiography has shown that a mainly dominant neurological affection was paresis of peroneal nerve in both lower extremities. In laboratory examination the titer of anti-myeloperoxidase anti-neutrophilic cytoplasmic antibodies (p-ANCA) was elevated. Due to renal involvement presented as a microscopic haematuria and decreasing of renal function, patient undergone kidney biopsy. It confirmed the immune vasculitis microscopic polyangiitis type with ANCA-associated glomerulonephritis. This is one of rare case of microscopic polyangiitis without lung simptomatology, first presented with asymmetrical polineuropathy of lower extremities. The patient was treated with methylprednisolone and cyclophosphamide in dosis adjusted to the level of disease severity and the renal function (methylprednisolone 1 mg/kg of body weight for two months with gradually tapering to the minimum effective dose and cyclophosphamide 1 mg/kg of body weight). This treatment lead to the partial remission of disease. In maintenance therapy azathioprin was introduced instead of cyclophosphamide.

A Case of Microscopic Polyangiitis Presented as Pleural Effusion

Microscopic polyangiitis is a necrotizing vasculitis, characterized by inflammation of small vessels (capillaries, venules, and arterioles) with few or no immune deposits. The kidneys are the most commonly affected organs and are involved in 90% of patients, whereas pulmonary involvement occurs in a minority of cases (10% to 30%). In cases of lung disease, diffuse alveolar hemorrhage with pulmonary capillaritis is the most common manifestation. Microscopic polyangiitis is strongly associated with antineutrophil cytoplasmic autoantibody, which is a useful diagnostic serological marker. We report a case of microscopic polyangiitis presented as pleural effusion in a 67-year-old female. Pleural effusions have been reported in some cases previously, but the number of cases were small and their characteristics have not been well described. This report describes characteristic findings of pleural fluid and its histological features in a case of microscopic polyangiitis.

A case of femoral hemorrhage in a patient with microscopic polyangiitis with low levels of myeloperoxidase-antineutrophil cytoplasmic autoantibody

We present the case of a 67-year-old female with femoral hemorrhage accompanied by microscopic polyangiitis. She was admitted to our hospital with symptoms of general fatigue, fever, and edema of the lower limbs. She was diagnosed with microscopic polyangiitis on the basis of the cardinal symptoms of the condition, including rapidly progressive glomerulonephritis and the presence of myeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA), albeit at a low titer. Renal biopsy demonstrated the presence of fibrocellular crescent-shaped glomeruli with interstitial infiltration. No immune deposits were detected in immunofluorescence studies. The patient was treated with steroids and anti-platelet agents; subsequently, the inflammatory reaction subsided and MPO-ANCA and C-reactive protein titers decreased. However, on day 14, the patient experienced sudden onset of swelling in the left femoral region accompanied by hypotension. Her hemoglobin level dropped from 8.8 to 4.5g/dl in the subsequent hours. Although computed tomography of the legs revealed an extensive hematoma in the left quadriceps femoris muscle, the patient recovered after receiving a transfusion and supportive therapy with discontinuation of dipyridamole. Thereafter, her renal function improved, and she was discharged. To our knowledge, this is the first report of a case of microscopic polyangiitis accompanied by femoral hemorrhage.

A Case of Microscopic Polyangiitis with Repeated Large Vessel Diseases

A Case of Microscopic Polyangiitis with Repeated Large Vessel Diseases

Microscopic polyangiitis initiated with liver dysfunction, calf pain and fever of unknown origin

We report herein a case of microscopic polyangiitis (MPA), presenting onset with a spiking fever, liver/biliary dysfunction without jaundice and calf pain without elevation of serum creatine phosphokinase. During 1 month of careful examinations for initial diagnosis, the patient developed renal dysfunction and pulmonary hemorrhage. Based on the results of positive MPO-ANCA, renal and pulmonary involvements, the patient was diagnosed with MPA and treated with high-dose prednisolone and oral cyclophosphamide. Soon after initiation of the treatment, symptoms such as fever, calf pain, liver/biliary dysfunction and renal dysfunction disappeared with decrease of MPO-ANCA titer to the normal level.

A Case of Microscopic Polyangiitis which Showed Spontaneous Disappearance of Purpura before Treatment

A Case of Microscopic Polyangiitis which Showed Spontaneous Disappearance of Purpura before Treatment

Pulmonary consolidation with fever is not always pneumonia: A case of microscopic polyangiitis and review of the literature

Microscopic polyangiitis is a non-granulomatous, systematic and small-vessel vasculitis in which necrotizing glomerulonephritis is very common and pulmonary capillaritis often occurs. We report a patient with fever, fatigue and dry cough who initially had been diagnosed as having pneumonia. A thoracic CT was performed showing widespread multiple nodular lesions, some with a central air-bronchogram and thickened bronchovascular bundles and some connected to nodules. No cavitation or ground glass opacities were observed. Fibreoptic bronchoscopy was normal and the cultures from the BAL were negative. Additionally, during his stay in the hospital his hematocrit was gradually falling, while the erythrocyte sedimentation rate increased. Urinanalysis revealed proteinuria and active urinary sediment. p-ANCA was positive and renal biopsy revealed focal pauci-immune necrotic glomerulonephritis. Pulmonary–renal syndrome was suspected and renal biopsy was performed. The diagnosis of microscopic polyangiitis was established and the patient began treatment with pulse intravenous methylprednisolone and cyclophosphamide. After induction of remission, the patient received maintenance therapy. Finally, an extensive review of the literature on microscopic polyangiitis is presented.

15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy.

The combination of cyclophosphamide (CYC) and oral corticosteroids is effective in the majority of patients with antineutrophil cytoplasmic antibody-associated vasculitis (AASV), but it carries substantial risk of drug-related morbidity and mortality. New regimens are desired, especially in refractory cases. The immunosuppressant 15-deoxyspergualin (DSG) is effective in experimental autoimmune disease and transplantation as well as in acute kidney transplant rejection in humans. To assess the efficacy and safety of DSG, an open label multicenter trial was conducted in patients with AASV who were either unresponsive or had contraindications for standard immunosuppressants. Included were 19 cases of Wegener granulomatosis and one case of microscopic polyangiitis. Nine of them had received CYC shortly before study entry without apparent therapeutic success. DSG (0.5 mg/kg per d) was given for 2 to 3 wk until the WBC count dropped to 3000/ micro l followed by a rest until at least a WBC of 4000/ micro l was reached again. This was repeated up to six cycles. During the study, no other immunosuppressants besides steroids were allowed. Disease improvement during treatment with DSG was achieved in 70% of cases (six cases of complete remission; eight cases of partial remission). Leucopenia occurred in each patient in a regular pattern during the cycles and was transient without exception. No mortality or septicemia was observed. Mild to moderate infections mainly in the respiratory tract were observed but resolved under adequate treatment without sequel. It is concluded that treatment with DSG is successful in patients with refractory Wegener granulomatosis under careful monitoring of WBC count.