爱肾分享 | Sarcoidosis

limingxin1969 2019-07-31

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AJKD Atlas of Renal Pathology: Sarcoidosis

Agnes B. Fogo, MD,1 Mark A. Lusco, MD,1 Behzad Najafian, MD,2 and Charles E. Alpers, MD2

Clinical and Pathologic Features

Sarcoidosis is a systemic disease of middle-aged patients, with an incidence of 15-20 per 100,000. African Americans are affected much more commonly than those of European ancestry, with the exception of increased incidence in patients of Scandinavian ancestry; there is a slight female preponderance. Organs most commonly involved include lung, lymph nodes (particularly hilar), eyes, and skin. Kidney disease develops in up to a third of patients, and may present as acute kidney injury related to hypercalcemia, or GFR decline and low-level proteinuria due to granulomatous parenchymal injury. Rarely, patients have associated membranous nephropathy, and then have marked proteinuria.

Light microscopy: Numerous interstitial noncaseating well-formed granulomas with multinucleated giant cells are present, and variable interstitial fibrosis and tubular atrophy. Calcifications may be present.

Immunofluorescence microscopy: Noncontributory (unless associated with membranous nephropathy, in which case, granular glomerular capillary loop deposits are present).

Electron microscopy: Noncontributory (unless associated with membranous nephropathy, in which case, subepithelial glomerular capillary loop deposits are present).

Etiology/Pathogenesis

Sarcoidosis is of unknown etiology and pathogenesis. Abnormal T-cell function, and a possible contribution of infection (eg, atypical mycobacteria), have been postulated. Increased 1,25-dihydroxyvitamin D3 from macrophages is thought to result in hypercalcemia.

Differential Diagnosis

Noncaseating granulomas are sometimes present with drug-induced hypersensitivity reactions, but are vaguely formed, and usually not numerous and confluent. Granulomas with central necrosis (caseating) are present with infection, such as fungus or tuberculosis, and special stains/culture/polymerase chain reaction amplification for these organisms then typically give positive results. Vague granulomas with fibrinoid necrosis, often involving extensively destroyed glomeruli and/or vascular structures, can be seen in pauci-immune necrotizing crescentic glomerulonephritis, and granulomas can also be encountered in anti–glomerular basement membrane antibody– mediated disease, usually surrounding glomeruli with crescents and necrosis.

Key Diagnostic Features

-Well-formed noncaseating granulomas in the interstitium

-Granulomas are numerous and confluent

Figure 1. Sarcoidosis in the kidney is characterized by sharply delineated nonnecrotizing granulomas with frequent epithelioidtype multinucleated giant cells with associated interstitial fibrosis and lymphoplasmacytic interstitial infiltrate (Jones stain). Reproduced with permission from AJKD 37(2):e12-e13. Continued

Figure 2. Sarcoidosis in the kidney with well-delineated nonnecrotizing granulomas with adjacent tubulointerstitial fibrosis (Jones stain). Reproduced with permission from AJKD 37(2):e12-e13.

Figure 3. Sarcoidosis in the kidney with nonnecrotizing granulomas comprising large giant cells with surrounding lymphoplasmacytic interstitial infiltrate and early tubulointerstitial fibrosis (Jones stain). Reproduced with permission from AJKD 37(2):e12-e13.

Figure 4. Sarcoidosis in the kidney with well-delineated granuloma with numerous giant cells surrounding an intact glomerulus (hematoxylin and eosin stain). Reproduced with permission from AJKD 37(2):e12-e13.

Figure 5. Sarcoidosis in the kidney, with multinucleated giant cells (periodic acid–Schiff stain). Reproduced with permission from AJKD 37(2):e12-e13. - Atlas of Renal Pathology II e6 Am J Kidney Dis. 2016;68(1):e5-e6

前期回顾：

Glomerular Manifestations of Systemic Diseases

1.Cryoglobulinemic Glomerulonephritis

2.AL Amyloidosis

3.Light and Heavy Chain Deposition Disease

4.Heavy Chain Deposition Disease

Primary Glomerular Diseases

1. Minimal Change Disease

2. Congenital Nephrotic Syndrome of Finnish Type

3.Diffuse Mesangial Sclerosis

4.Tip Lesion Variant of Focal Segmental Glomerulosclerosis

5.Collapsing Glomerulopathy