After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support

After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support. the Japanese Society of Nephrology also recommends daily treatment with oral cyclophosphamide (25-100 mg/day) or intravenous pulses of cyclophosphamide (250-750 mg/m2) with corticosteroids (5). Initial therapy with corticosteroids alone is indicated for cases in which aggressive treatment is required; however, the use of immunosuppressive agents is not desirable, particularly in patients MT-7716 hydrochloride older than 70 years. Diffuse alveolar hemorrhage (DAH) is well known as a serious AAV symptom associated with high mortality (6,7). In a study of 107 patients with biopsy-proven ANCA-associated microscopic polyangiitis and glomerulonephritis, the relative risk of death was 8.65 times higher in patients with pulmonary hemorrhage than in those without (7). However, the adverse effects of this therapy cannot be overlooked, and glucocorticoids and immunosuppressive agents may increase the susceptibility to infection, which is MT-7716 hydrochloride a strong indicator of mortality in patients with AAV (8). Plasma exchange is a promising option for the treatment of AAV and is recommended in the guidelines (3,5,9). Because ANCA causes necrotizing glomerulonephritis and small vessel vasculitis, the removal of ANCA may contribute to the control of disease activity and the prevention of organ damage. Although the guidelines recommend the addition of plasma exchange to corticosteroid and cyclophosphamide therapy in patients with advanced kidney dysfunction, whether or not plasma exchange is effective in patients with alveolar hemorrhage is unclear (10). We herein present the case of a 76-year-old woman with severe Rabbit Polyclonal to p55CDC AAV and DAH who successfully achieved remission following prompt initiation of plasma exchange and low-dose prednisolone. Case Report A 76-year-old woman was admitted to our hospital with shortness of breath. One year before admission, she had presented with a three-month history of dry cough and was admitted to a local hospital for an examination. Chest-X ray showed ground glass opacity, and respiratory function testing showed an obstructive pattern. Macrophages were observed in the bronchoalveolar lavage fluid. She was diagnosed with idiopathic pulmonary fibrosis and discharged with home oxygen therapy only when she experienced shortness of breath. At the time, her serum creatinine level was 0.56 mg/dL, and there were MT-7716 hydrochloride no abnormalities on urinalysis. Three months before admission, she was again admitted to the local hospital for shortness of breath, anorexia, and weight loss (15 kg over 3 months), and her serum creatinine level was elevated to 2.23 mg/dL. In addition, urinary protein, hematuria (20-29 per high-power field), and markedly elevated myeloperoxidase (MPO)-ANCA were detected. She was transferred to our hospital for further examination and treatment of suspected MPO-ANCA-associated glomerulonephritis. On admission, a physical examination revealed fine crackles, pale conjunctivae, clubbed fingers, MT-7716 hydrochloride body weight of 62.0 kg, body temperature of 36.7, blood pressure of 132/60 mmHg, pulse rate of 80 beats/min, percutaneous oxygen saturation of 90% on atmospheric air with a respiratory rate of 12-16 breath/min, and her Birmingham Vasculitis Activity Score was 24. As shown in Table, the serum creatinine level was 2.35 mg/dL, and the MPO-ANCA titer was 336 U/mL (normal range, 0-3.5 U/mL); normocytic anemia was detected. Table. Laboratory Findings on Admission. Complete Blood CountAPTT (second)26.8White blood cell (L)8,800PT (%)75.6Neutrophil (%)69.2Fibrinogen (mg/dL)458Eosinophil (%)3.6Antinuclear antibody (dilution)40Monocyte (%)3.8C3 complement (mg/dL)98Basophil (%)0.4C4 complement (mg/dL)13.9Lymphocyte (%)23.0PR3-ANCA (U/mL)0.5Hemoglobin (g/dL)8.7MPO-ANCA (U/mL)336.0Hematocrit (%)28.8Anti-GBM antibody(-)MCV(fL)95IgG (mg/dL)2,175MCH (pg)28.6IgA (mg/dL)317MCHC (%)30.2IgM (mg/dL)83Platelet (104/L)31Arterial Blood Gas (O2 3L/min)Serum ChemistrypH7.425Urea nitrogen (mg/dL)30.2 pO2 (mmHg)104.0Creatinine (mg/dL)2.35 pCO2 (mmHg)34.8eGFR (mL/min/1.73 m2)16.2HCO3 (mEq/L)22.4Sodium (mEq/L)137Base excess (mEq/L)10.6Potassium (mEq/L)4.5Anion Gap (mEq/L)7.5Chloride (mEq/L)104UrinalysisCalcium (mg/dL)8.7Gravity1.009Phosphorus (mg/dL)4.5pH5.5C-reactive protein (mg/dL)5.84Proteinuria2+Fe (g/dL)24UPCR (g/gCr)1.55TIBC (g/dL)210Hematuria3+Ferritin (ng/mL)454Red blood cell (/HPF)10-19White blood cell (/HPF)10-19Granular cast2+ Open in a separate window MCV: mean corpuscular volume, MCH: mean corpuscular hemoglobin, MCHC: mean corpuscular hemoglobin concentration, eGFR: estimated glomerular filtration rate, TIBC: total iron binding capacity, APTT: activated partial thromboplastin time, PT: prothrombin time, PR3: proteinase-3, MPO: myeloperoxidase, ANCA: antineutrophil cytoplasmic autoantibody, GBM: glomerular basement membrane, RBC: red blood cell, UPCR: urinary protein to MT-7716 hydrochloride creatinine ratio On the day following admission, percutaneous renal biopsy was performed (Fig. 1). The histopathological findings revealed cellular crescents in 5 out of 10 glomeruli. No glomeruli showed mesangial proliferation or basement membrane changes. Tubulitis was found in.

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