She was also found to have extensive DVT of the lower extremities
She was also found to have extensive DVT of the lower extremities. an ADAMTS13 level of 62%, a rheumatoid factor level of 151.7 IU/L, and myeloperoxidase (MPO)-ANCA level of 173 AU/mL. A computed tomography scan of the chest/abdomen/pelvis revealed pulmonary fibrosis and multifocal consolidations. She was also found to CPI 455 have extensive DVT of the lower extremities. Renal biopsy revealed early changes of TMA with one cellular crescent. She was diagnosed with AAV based on the kidney and lung findings, as well as the high titer MPO-ANCA. Her platelet count and creatinine improved significantly following treatment with plasma exchange, steroids, and rituximab. Unfortunately, she was then found to have an acute bowel perforation and expired. Even though typically rare, an increased incidence of venous thromboembolism (VTE) and TMA has been reported in patients with AAV. Its prompt recognition and treatment by clinicians are critical to mitigate the unfavorable outcomes from this condition. O157:H7 testing was not feasible as this patient did not have a bowel movement during hospital admission. She was later found to have an extensive lower extremity DVT, and heparin was administered. A workup for malignancy was ordered along with computed tomography (CT) scan of the chest/abdomen/pelvis. The CT scan revealed a pulmonary fibrosis pattern and multifocal areas of consolidation in the upper lung (Figure ?(Figure1).1). Tumor markers (CEA, CA 19-9, CA 125, AFP) were negative. Rituximab was started as the patients ANCA results were positive. Her platelet count and creatinine levels improved significantly following treatment (Table ?(Table22). Table 1 The patients initial workupADAMTS13: a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; AFP: alpha-fetoprotein; ANA: antinuclear antibody; ANCA: antineutrophil cytoplasmic antibodies; aPTT: activated partial thromboplastin time; CA: cancer antigen; CEA: carcinoembryonic antigen; CRP: C-reactive protein; ENA: extractable nuclear antigen; ESR: erythrocyte sedimentation rate; GBM: glomerular basement membrane; HBsAg: hepatitis B surface antigen; Hb: hemoglobin; HCV: hepatitis C virus; HIV: human immunodeficiency virus; LDH: lactate dehydrogenase; MPO: myeloperoxidase antibodies; PT: prothrombin time. Test nameReference rangeLab valueHematology?Hb (g/dL)12.0 – 16.014.5???Platelet count (103/L)130 – 40018???Haptoglobin (mg/dL)30 – 200 20???LDH CPI 455 (U/L)313 – 6181627.5???D-Dimer, Quant (g/mL)0.00 – 0.50 20.00? ?Fibrinogen (mg/dL)214 – 48192?? aPTT (seconds)23 – 3749?? PT11.3 – 14.719.1?? INR0.9 – 1.21.6Rheumatology panel?? ANA titerNegative at 1:401:80? ?ENA antibodies screen (EU)20.0004.123?? Anti-DNA antibody (IU)25.00014.220?? Rheumatoid factor (IU/mL)12.0151.7?? Cryoglobulin quant, bloodNegativeNegative?? GBM Ab IgG (AU/mL)0 – 190?? Sm antibody (AU/mL)0 – 405?? Anti-Scl70 IgG (AU/mL)0 – 400?? Myositis antibodyNegativeNegative?? ANCA IgG 1:201:1280?? MPO-ANCA (AU/mL)0 – 19173?? PR3-ANCA (AU/mL)0 – 191?? Direct antiglobulin test?Negative? ?ADAMTS1350-160%62%Inflammatory markers?? Procalcitonin (ng/mL) 0.250.28?? CRP (mg/L)1031?? ESR (mm)0 – 201?? C3 (mg/dL)88 – 165121?? C4 (mg/dL)14 – 4427Infectious workup?? HIV Ab?Negative?? HCV Ab?Negative?? HbsAg?Negative?? Blood culture?No growth at five daysTumor markers?? CEA (ng/mL)3.01.0?? CA 19-9 (U/mL)37.0018.20?? CA 125 (U/mL)3512?? AFP (ng/mL) 7.512.11Others?? Vitamin B12 (pg/mL)211 – 911908?? Folate (ng/mL)3.0 – 20.017.6 Open in a separate window Open in CPI 455 a separate window Figure 1 Computed tomography scan of the chest/abdomen/pelvis showed pulmonary fibrosis pattern, small bilateral perfusion, and multifocal areas of consolidation Table 2 Kidney-function-and-platelet-level-monitoring-during-the-patients-length-of-stay Test nameReference rangeDay 1Day 2Day 3Day 4Day 5Day 6Day 7Creatinine (mg/dL)0.7 – 1.38.356.924.903.462.962.201.80Platelet ( 103/L)130 – 40018942108127112226 Open in a separate window A kidney biopsy was done following the resolution of her thrombocytopenia. Light microscopic examination of the kidney specimen revealed 23 glomeruli, of which six were globally sclerotic, one demonstrated early glomerular capillary thrombosis, and another demonstrated a cellular crescent (Figure ?(Figure2).2). There was no evidence of vasculitis, but moderate arteriosclerosis was present. Mild interstitial fibrosis and tubular atrophy were found in the tubulointerstitium, involving 10% to 15% of the renal cortical tissue. Electron microscopy showed no electron-dense immune-type deposits or organized deposits. Immunofluorescent staining (IgG, IgA, IgM, C3, C1q, kappa, and lambda) of the glomeruli were negative. Open in a separate window Figure 2 Kidney biopsy showed glomerulus with a cellular crescent (H&E stain) During the course of treatment, this patient developed an acute bowel perforation not amenable to treatment and expired. Discussion Our patient was admitted with TMA and DVT. At first, malignancy was an obvious option for the differential diagnoses; however, the workup was negative. An infection workup was also negative. This patient was diagnosed with AAV based on the kidney and lung findings as well as the very high titer of MPO-ANCA, consistent with microscopic polyangiitis (MPA). Pulmonary involvement is observed in 25% of patients with MPA, including diffuse alveolar hemorrhage, interstitial lung disease (ILD), and pleuritis [1,2]. ILD Rabbit polyclonal to FABP3 is present in about 7.2% of patients at the time of MPA diagnosis [1]. Approximately 80% of patients with MPA have renal manifestations [2]. Global glomerulosclerosis (defined as sclerotic changes in the glomerulus that compose 80%.