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Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis.

Ward ND, Cosner DE, Lamb CA, Li W, Macknis JK, Rooney MT, Zhang PL - Case Rep Pathol (2014)

Bottom Line: A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody.The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination.Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Anatomic Pathology, William Beaumont Hospital, 3601 W 13 Mile Road, Royal Oak, MI 48073, USA.

ABSTRACT
A rat model of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

No MeSH data available.


Related in: MedlinePlus

ANCA associated vasculitides in a 64-year-old man. In the wedge lung biopsy, there was diffuse alveolar hemorrhage (a). The hemosiderosis (hemosiderin-laden macrophages) was confirmed by positive iron staining in (b) (magnification ×100 for both (a) and (b)). High power view in the lung showed focal giant cell (c) and neutrophil aggregate and margination along the endothelium of an artery (d). Renal biopsy revealed cellular crescent formation (e) and focal fibrinoid arteritis (f). Magnification ×400 in (c)–(f).
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fig1: ANCA associated vasculitides in a 64-year-old man. In the wedge lung biopsy, there was diffuse alveolar hemorrhage (a). The hemosiderosis (hemosiderin-laden macrophages) was confirmed by positive iron staining in (b) (magnification ×100 for both (a) and (b)). High power view in the lung showed focal giant cell (c) and neutrophil aggregate and margination along the endothelium of an artery (d). Renal biopsy revealed cellular crescent formation (e) and focal fibrinoid arteritis (f). Magnification ×400 in (c)–(f).

Mentions: A 64-year-old male had progressive dyspnea over six-month duration. The patient had a history of asthma but never smoked. A recent cardiac evaluation included normal echocardiogram and stress test. Computerized tomography (CT) chest imaging without contrast revealed bilateral mild ground-glass opacities in the lungs, slightly greater within the middle and lower lobes on the right side, suggestive of mild interstitial lung disease. He was found to have positive serum p-ANCA at titer of 1 : 640 while the antiglomerular basement membrane antibody was negative. His serum test for myeloperoxidase was positive at greater than 8 units (normal < 0.4 unit) while his serum level of proteinase-3 was negative. Wedge lung biopsies from the right upper, middle, and lower lobes were performed to reveal red to purple color from pleural surface and red color on the cross sections grossly. Microscopically, the biopsies mainly revealed diffuse hemorrhage and hemosiderosis (iron positive macrophages in alveoli) (Figures 1(a) and 1(b)), but no definite vasculitis was present. The case was sent for expert opinion with a diagnosis of idiopathic pulmonary hemosiderosis. They commented that (1) findings were consistent with a diffuse alveolar hemorrhage syndrome; (2) the absence of vasculitis and capillaritis argued against the most common pulmonary vasculitides, namely, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis; and (3) Goodpasture's syndrome was possible but required the identification of antiglomerular basement membrane antibodies. One month later, because the lung biopsies were not conclusive and the patient showed repeated positivity for ANCA while a mild elevation of serum creatinine (1.23 mg/dL) and mild hematuria was present, then a renal biopsy was done to rule out ANCA associated crescentic glomerulonephritis.


Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis.

Ward ND, Cosner DE, Lamb CA, Li W, Macknis JK, Rooney MT, Zhang PL - Case Rep Pathol (2014)

ANCA associated vasculitides in a 64-year-old man. In the wedge lung biopsy, there was diffuse alveolar hemorrhage (a). The hemosiderosis (hemosiderin-laden macrophages) was confirmed by positive iron staining in (b) (magnification ×100 for both (a) and (b)). High power view in the lung showed focal giant cell (c) and neutrophil aggregate and margination along the endothelium of an artery (d). Renal biopsy revealed cellular crescent formation (e) and focal fibrinoid arteritis (f). Magnification ×400 in (c)–(f).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4265693&req=5

fig1: ANCA associated vasculitides in a 64-year-old man. In the wedge lung biopsy, there was diffuse alveolar hemorrhage (a). The hemosiderosis (hemosiderin-laden macrophages) was confirmed by positive iron staining in (b) (magnification ×100 for both (a) and (b)). High power view in the lung showed focal giant cell (c) and neutrophil aggregate and margination along the endothelium of an artery (d). Renal biopsy revealed cellular crescent formation (e) and focal fibrinoid arteritis (f). Magnification ×400 in (c)–(f).
Mentions: A 64-year-old male had progressive dyspnea over six-month duration. The patient had a history of asthma but never smoked. A recent cardiac evaluation included normal echocardiogram and stress test. Computerized tomography (CT) chest imaging without contrast revealed bilateral mild ground-glass opacities in the lungs, slightly greater within the middle and lower lobes on the right side, suggestive of mild interstitial lung disease. He was found to have positive serum p-ANCA at titer of 1 : 640 while the antiglomerular basement membrane antibody was negative. His serum test for myeloperoxidase was positive at greater than 8 units (normal < 0.4 unit) while his serum level of proteinase-3 was negative. Wedge lung biopsies from the right upper, middle, and lower lobes were performed to reveal red to purple color from pleural surface and red color on the cross sections grossly. Microscopically, the biopsies mainly revealed diffuse hemorrhage and hemosiderosis (iron positive macrophages in alveoli) (Figures 1(a) and 1(b)), but no definite vasculitis was present. The case was sent for expert opinion with a diagnosis of idiopathic pulmonary hemosiderosis. They commented that (1) findings were consistent with a diffuse alveolar hemorrhage syndrome; (2) the absence of vasculitis and capillaritis argued against the most common pulmonary vasculitides, namely, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis; and (3) Goodpasture's syndrome was possible but required the identification of antiglomerular basement membrane antibodies. One month later, because the lung biopsies were not conclusive and the patient showed repeated positivity for ANCA while a mild elevation of serum creatinine (1.23 mg/dL) and mild hematuria was present, then a renal biopsy was done to rule out ANCA associated crescentic glomerulonephritis.

Bottom Line: A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody.The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination.Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Anatomic Pathology, William Beaumont Hospital, 3601 W 13 Mile Road, Royal Oak, MI 48073, USA.

ABSTRACT
A rat model of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

No MeSH data available.


Related in: MedlinePlus