Background
Renal biopsy findings | Connective tissue disease |
---|---|
Tubulointerstitial nephritis (TIN)
| |
Mesangial proliferative glomerulonephritis/IgA nephropathy
| |
Focal segmental glomerulosclerosis (FSGS)
| |
Cryoglobulinemic membrano-proliferative glomerulonephritis
| |
Minimal change disease
| |
Membranous nephropathy
| |
Secondary renal amyloidosis
| |
Thrombotic microangiopathy
| |
Diffuse proliferative glomerulonephritis
| Dermatomyositis [49] |
IgM nephropathy
| Systemic lupus erythematosus [81] |
Collapsing glomerulopathy
| Systemic lupus erythematosus [79] |
Lupus nephritis
| |
Fibrillary glomerulonephritis
| |
Necrotizing crescentic glomerulonephritis (including drug-induced forms)
| |
Focal proliferative glomerulonephritis
| Rheumatoid arthritis [17] |
Crescentic GN with FSGS
| Polymyositis [61] |
C3 nephropathy
| Antiphospholipid syndrome [124] |
Review
Sjögren syndrome
Introduction
Histopathology/kidney involvement
Therapy
Conclusion
Scleroderma renal crisis
Introduction
Histopathology/kidney involvement
Therapy
Conclusion
Dermatomyositis and polymyositis
Introduction
Histopathology/kidney involvement
Therapy
Conclusion
Systemic lupus erythematosus
Introduction
1. Malar rash | Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds |
2. Discoid rash | Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions |
3. Photosensitivity | Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation |
4. Oral ulcers | Oral or nasopharyngeal ulceration, usually painless, observed by physician |
5. Non-erosive arthritis | Involving two or more peripheral joints, characterized by tenderness, swelling or effusion |
6. Pleuritis/Pericarditis | 1. Pleuritis, convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion, or |
2. Pericarditis, documented by electrocardiogram or rub or evidence of pericardial effusion | |
7. Renal disorder | 1. Persistent proteinuria >0.5 grams per day or more than 3+ on urine dipstick testing, or |
2. Cellular casts (may be red cell, hemoglobin, granular, tubular, or mixed) | |
8. Neurologic disorder | 1. Seizures, in the absence of offending drugs or known metabolic derangements; for example, uremia, ketoacidosis, or electrolye imbalance, or |
2. Psychosis, in the absence of offending drugs or known metabolic derangements; for example, uremia, ketoacidosis, or electrolyte imbalance | |
9. Hematologic disorder | 1. Hemolytic anemia with reticulocytosis, or |
2. Leukopenia <4.000/mm3 on ≥2 occasions, or | |
3. Lymphopenia <1.500/mm3 on ≥2 occasions, or | |
4. Thrombocytopenia <100.000/mm3 in the absence of offending drugs | |
10. Immunologic disorder | 1. Anti-DNA: antibody to native DNA in abnormal titer, or |
2. Anti-Sm: presence to antibody of SM nuclear antigen, or | |
3. Positive finding of antiphospholipid antibodies on: | |
● An abnormal serum level of IgG or IgM anticardiolipin antibodies | |
● A positive test result for lupus anticoagulant using a standard method, or | |
● A false-positive test result for at least six months confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test | |
11. Positive anti-nuclear antibody | An abnormal titer of anti- nuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs |
Histopathology/kidney involvement
Class I
|
Minimal mesangial lupus nephritis
|
Normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence | |
Class II
|
Mesangial proliferative lupus nephritis
|
Purely mesangial hypercellularity of any degree or mesangial matrix expansion by light microscopy, with mesangial immune deposits | |
May be a few isolated subepithelial deposits visible by immunofluorescence or electron microscopy, but not by light microscopy | |
Class III
|
Focal lupus nephritis
|
Active or inactive focal, segmental or global endo- or extracapillary glomerulonephritis involving <50% of all glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations | |
Class III (A)
| Active lesions: focal proliferative lupus nephritis |
Class III (A/C)
| Active and chronic lesions: focal proliferative and sclerosing lupus nephritis |
Class III (C)
| Chronic inactive lesions with glomerular scars: focal sclerosing lupus nephritis |
Class IV
|
Diffuse lupus nephritis
|
Active or inactive diffuse, segmental or global endo- or extracapillary glomerulonephritis involving ≥50% of all glomeruli, typically with diffuse subendothelial immune deposits, with or without mesangial alterations. This class is divided into diffuse segmental (IV-S) lupus nephritis when ≥50% of the involved glomeruli have segmental lesions, and diffuse global (IV-G) lupus nephritis when ≥50% of the involved glomeruli have global lesions. Segmental is defined as a glomerular lesion that involves less than half of the glomerular tuft. This class includes cases with diffuse wire loop deposits but with little or no glomerular proliferation | |
Class IV-S (A)
| Active lesions: diffuse segmental proliferative lupus nephritis |
Class IV-G (A)
| Active lesions: diffuse global proliferative lupus nephritis |
Class IV-S (A/C)
| Active and chronic lesions: diffuse segmental proliferative and sclerosing lupus nephritis |
Active and chronic lesions: diffuse global proliferative and sclerosing lupus nephritis | |
Class IV-S (C)
| Chronic active lesions with scars: diffuse segmental sclerosing lupus nephritis |
Class IV-G (C)
| Chronic inactive lesions with scars: diffuse global sclerosing lupus nephritis |
Class V
|
Membranous lupus nephritis
|
Global or segmental subepithelial immune deposits or their morphologic sequelae by light microscopy and by immunofluorescence or electron microscopy, with our without mesangial alterations | |
Class V lupus nephritis may occur in combination with class III or IV in which case both will be diagnosed | |
Class V lupus nephritis show advanced sclerosis | |
Class VI
|
Advanced sclerosis lupus nephritis
|
≥90% of glomeruli globally sclerosed without residual activity |
Therapy
Conclusion
Kidney disease in antiphospholipid syndrome
Introduction
Histopathology/kidney involvement
Therapy
Conclusion
Rheumatoid arthritis
Introduction
Histopathology/kidney involvement
Therapy
Conclusion
Conclusion and future directions
Biopsy indication | rapid deterioration of renal function (exclude post renal and pre renal disorders first) |
Biopsy indication | proteinuria >1 g/d (measured by collecting urine; collection over the course of a 24-hour period; to begin urine collection, the patient voids and discards the urine already in the bladder, afterwards urine for the next 24 hours has to be collected to ensure accurate results), if other causes of proteinuria are ruled out |
the EULAR/ERA-EDTA recommendations for the management of lupus nephritis suggest performing a renal biopsy if reproducible proteinuria >0.5 g/d is present (especially with glomerular hematuria and/or cellular cases) [72] | |
Biopsy indication | nephritic urine sediment (red blood cell casts) with deterioration of kidney function (estimated GFR <60 ml/min) if pre-existing impaired renal function is ruled out |
Consider re-biopsy | increase in proteinuria/serum creatinine despite ongoing immunosuppressive therapy (exclude post-renal and pre-renal disorders first); consider a repeat kidney biopsy due to potential phenotype change (for example, lupus nephritis) |
Biopsy indication | suspected interstitial nephritis, findings of white blood cell casts; leukocyturia (due to proton pump inhibitors, non-steroidal anti-rheumatic drugs, Sjögren syndrome, rheumatoid arthritis, and so on) |
Biopsy indication | diagnostic approach in case of uncertainties, when kidney involvement is suspected, but absolute indications are not met |