出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/08/23 06:14:44」(JST)
| Lupus nephritis | |
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| Classification and external resources | |
Micrograph of diffuse proliferative lupus nephritis showing increased mesangial matrix and mesangial hypercellularity. Kidney biopsy. PAS stain.
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| ICD-10 | M32.1+N08.5* |
| ICD-9 | 583.81 |
| MedlinePlus | 000481 |
| eMedicine | med/1597 |
| MeSH | D008181 |
Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. Apart from the kidneys, SLE can also damage the skin, joints, nervous system and virtually any organ or system in the body.
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General symptoms of lupus include malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, pleuropericarditis, renal disease, neurological manifestations, and haematological disorders.
Clinically, SLE usually presents with fever, weight loss (100%), arthralgias, synovitis, arthritis (95%), pleuritis, pericarditis (80%), malar facial rash, photodermatosis, alopecia (75%), anaemia, leukopaenia, thrombocytopaenia, and thromboses (50%).
About half of cases of SLE demonstrate signs of lupus nephritis at one time or another. Renal-specific signs include proteinuria (100%), nephrotic syndrome (55%), granular casts (30%), red cell casts (10%), microhematuria (80%), macrohematuria (2%), reduced renal function (60%), RPGN (30%), ARF (2%), hypertension (35%), hyperkalemia (15%) and tubular abnormalities (70%).
In histology, stage I (minimal mesangial) disease has a normal appearance under light microscopy, but mesangial deposits are visible under electron microscopy. At this stage urinalysis is typically normal.
stage II disease (mesangial proliferative) is noted by mesangial hypercellularity and matrix expansion. Microscopic haematuria with or without proteinuria may be seen. Hypertension, nephrotic syndrome, and acute renal insufficiency are rare at this stage.
stage III disease (focal lupus nephritis) is indicated by sclerotic lesions involving less than 50% of the glomeruli, which can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions. Under electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present. Immunofluorescence reveals the so-called "Full House" stain, staining positively for IgG, IgA, IgM, C3, and C1q. Clinically, haematuria and proteinuria is present, with or without nephrotic syndrome, hypertension, and elevated serum creatinine.
stage IV lupus nephritis (diffuse proliferative) is both the most severe, and the most common subtype. More than 50% of glomeruli are involved. Lesions can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions. Under electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present. Immunofluorescence reveals the so-called "Full House" stain, staining positively for IgG, IgA, IgM, C3, and C1q. Clinically, haematuria and proteinuria are present, frequently with nephrotic syndrome, hypertension, hypocomplementemia, elevated anti-dsDNA titres and elevated serum creatinine.
A wire-loop lesion may be present in stage III and IV. This is a glomerular capillary loop with subendothelial immune complex deposition that is circumferential around the loop. Stage V is denoted by a uniformly thickened, eosinophilic basement membrane. Stage III and IV are differentiated only by the number of glomeruli involved (which is subject to inherent sample bias), but clinically the presentation and prognosis are both expected to be more severe in stage IV versus stage III.
Stage V (membranous lupus nephritis) is characterized by diffuse thickening of the glomerular capillary wall (segmentally or globally), with diffuse membrane thickening, and subepithelial deposits seen under electron microscopy. Clinically, stage V presents with signs of nephrotic syndrome. Microscopic haematuria and hypertension may also been seen. Plasma creatinine is usually normal or slightly elevated, and stage V may not present with any other clinical/serological manifestations of SLE (complement levels may be normal; anti-DNA Ab may not be detectable). Stage V also predisposes the affected individual to thrombotic complications such as renal vein thromboses or pulmonary emboli.
A final stage is usually included by most practitioners, stage VI, or advanced sclerosing lupus nephritis. It is represented by Global sclerosis involving more than 90% of glomeruli, and represents healing of prior inflammatory injury. Active glomerulonephritis is usually not present. This stage is characterised by slowly progressive renal dysfunction, with relatively bland urine sediment. Response to immunotherapy is usually poor.
A tubuloreticular inclusion is also characteristic of lupus nephritis, and can be seen under electron microscopy in all stages. It is not diagnostic however, as it exists in other conditions. It is thought to be due to chronic interferon exposure.
The diagnosis of lupus nephritis depends on blood tests, urinalysis, X-rays, ultrasound scans of the kidneys, and a kidney biopsy. On urinalysis, a nephritic picture is found and RBC casts, RBCs and protenuria is found.
The World Health Organization has divided lupus nephritis into five stages based on the biopsy. This classification was defined in 1982 and revised in 1995.[1]
Medicines are prescribed that decrease swelling, lower blood pressure, and decrease inflammation by suppressing the immune system: Patients may need to monitor intake of protein, sodium, and potassium. Patients with severe disease should restrict their sodium intake to 2 grams per day and limit fluid as well. Depending on the histology, renal function and degree of proteinuria, patients may require steroid therapy or chemotherapy regimens such as cyclophosphamide, azathioprine, mycophenolate mofetil, or cyclosporine.
The medical therapy for lupus nephritis depends on the severity of the disease. For mild disease, corticosteroids are, in general, prescribed. More severe disease requires treatment with immunosuppressant agents. The two most commonly used agents are mycophenolate mofetil and intravenous cyclophosphamide. One recent study compared these two drugs.[3] The authors showed that patients with Class III or IV disease are more likely to benefit from mycophenolate mofetil as compared to cyclophosphamide. However, a larger study by the same authors that directly compared these therapies did not show that Mycophenolate was superiour to cyclophosphamide except in non-caucasian non-Asian patients [1]. In caucasian or Asian patients both treatments worked equally well. Both agents are associated with significant adverse effects; cyclophosphamide may induce permanent infertility in young women, and mycophenolate mofetil is associated with a higher risk of infection-related death.[4] One study concluded that in cases where lupus-related thrombotic thrombocytopenic purpura is present, plasmapheresis is life-saving, and must be instituted early to avoid a poor outcome.[5]
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| リンク元 | 「ループス腎炎」「LGN」「LN」 |
| 関連記事 | 「nephritis」「lupus」 |
I型 ：正常糸球体 normal II型 ：メサンギウム増殖性糸球体腎炎 mesangial alterations III型：巣状分節状糸球体腎炎 focal GN IV型 ：びまん性増殖性糸球体腎炎 diffuse proliferative GN V型 ：びまん性膜性糸球体腎炎 diffuse membranous GN VI型 ：硬化性糸球体腎炎 advanced sclerosing GN