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Glomerulonephritis, Rapidly Progressive Last Updated: April 11, 2006 |
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| Synonyms and related keywords: RPGN, rapidly progressive glomerulonephritis, Wegener granulomatosis, WG, Wegener's granulomatosis, Churg-Strauss disease, CS, Churg-Strauss syndrome, CSS, CSD, microscopic polyangiitis, MPA, circulating antineutrophil cytoplasmic antibody associated glomerulonephritis, ANCA disease, ANCA-associated disease, ANCA glomerulonephritis, ANCA-associated vasculitis, kidney disease, renal disease, kidney disorder, renal disorder, fibrinoid necrosis, extensive crescent formation, pauci-immune disease, pauciimmune disease, renal-limited necrotizing crescentic glomerulonephritis, NCGN, systemic lupus erythematosus, SLE, inflammatory bowel disease, IBD, sclerosing cholangitis, autoimmune hepatitis, rheumatoid arthritis, RA, Felty syndrome, Felty's syndrome, ANCA-associated vasculitides
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AUTHOR INFORMATION
| Section 1 of 9   |
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| Author: Kerry C Owens, MD, Consulting Staff, Department of Internal Medicine, Section of Nephrology, Integris Baptist Medical Center of Oklahoma City |
| Kerry C Owens, MD, is a member of the following medical societies:
American College of Physicians,
American Medical Association,
American Society of Nephrology,
International Society of Nephrology,
Oklahoma State Medical Association, and
Sigma Xi |
| Editor(s): F John Gennari, MD, Director, Division of Nephrology, Associate Chair for Academic Affairs, Robert F and Genevieve B Patrick Professor, Department of Medicine, University of Vermont College of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
George R Aronoff, MD, Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine;
Rebecca J Schmidt, DO, FACP, FASN, Clinical Associate Professor of Medicine, West Virginia School of Osteopathic Medicine; Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine;
and Vecihi Batuman, MD, FACP, FASN, Professor of Medicine, Chief, Section of Nephrology, Tulane University School of Medicine; Professor, Renal-Hypertension Section, Department of Medicine, Tulane University Medical Center and Veterans Affairs Medical Center |
Disclosure
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INTRODUCTION
| Section 2 of 9   |
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Background: Rapidly progressive glomerulonephritis (RPGN) is a disease of the kidney that results in a rapid decrease in the glomerular filtration rate of at least 50% over a short period, from a few days to 3 months. The main pathologic finding is fibrinoid necrosis (>90% of biopsy specimens); extensive crescent formation is present in at least 50% of glomeruli.
The term RPGN was first used to describe a group of patients who had an unusually fulminant poststreptococcal glomerulonephritis and a poor clinical outcome. Several years later, the antiglomerular basement membrane (anti-GBM) antibody was discovered to produce a crescentic glomerulonephritis in sheep, and, following this, the role of anti-GBM antibody in Goodpasture syndrome was elucidated. Soon afterwards, the role of the antibody in RPGN associated with Goodpasture disease was established.
In the mid 1970s, a group of patients was described who fit the clinical criteria for RPGN but in whom no cause could be established. Many of these cases were associated with systemic signs of vascular inflammation (systemic vasculitis), but some were characterized only by renal disease. A distinct feature of these cases was the virtual absence of antibody deposition after immunofluorescence staining of the biopsy specimens, which led to the label pauci-immune RPGN. More than 80% of patients with pauci-immune RPGN were subsequently found to have circulating antineutrophil cytoplasmic antibodies (ANCA), and thus, this form of RPGN is now termed ANCA-associated vasculitis.
RPGN is classified pathologically into 3 categories: (1) anti-GBM antibody disease (approximately 3% of cases), (2) immune complex disease (45% of cases), and (3) pauci-immune disease (50% of cases). The disorders are also classified based on their clinical presentation. Finally, they are classified immunologically, based on the presence or absence of ANCA. Below is a classification based on pathology, with the clinical syndromes and ANCA status subsumed under each pathological description.
Anti-GBM antibody
- Goodpasture syndrome
- Anti-GBM disease (only kidney involvement)
- Note: From 10-40% may have positive ANCA findings.
Immune complex
- Postinfectious
- Poststreptococcal
- Visceral abscess
- Collagen-vascular disease
- Lupus nephritis
- Henoch-Schönlein purpura
- Mixed cryoglobulinemia
- Primary renal disease
- Immunoglobulin A nephropathy
- Membranoproliferative glomerulonephritis
- Idiopathic
- Note: These may be perinuclear ANCA (pANCA)–positive without myeloperoxidase (MPO) specificity.
Pauci-immune
- Wegener granulomatosis (WG)
- Microscopic polyangiitis (MPA)
- Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
- Churg-Strauss syndrome
- Note: From 80-90% are ANCA-positive.
The conditions listed under the Anti-GBM antibody and Immune complex headings are discussed in other articles. The remainder of this article addresses the ANCA-associated diseases.
In 1982, Davies et al first noted the presence of ANCA in 8 patients with pauci-immune RPGN and systemic vasculitis. Hall et al noted this again in 1984, in 4 patients with a small vessel vasculitis. Subsequently, ANCA positivity was found to correlate closely with the clinical syndromes of WG, Churg-Strauss syndrome, and MPA. Pathophysiology: The link between ANCA and the pathogenesis of ANCA-associated disease is unknown, but it is postulated that neutrophils and mononuclear phagocytes are directly activated by ANCA and these activated cells, in turn, attack vessel walls, producing injury similar to that produced by anti-GBM antibodies or immune complexes.
ANCAs react with antigens in the primary granules in the cytoplasm of neutrophils (antiproteinase-3 [PR3]) and in lysosomes of monocytes (MPO). ANCA demonstrates 2 major types of staining patterns. Cytoplasmic ANCA (cANCA) produces a cytoplasmic staining pattern with central accentuation in alcohol-fixed neutrophils. pANCA demonstrates a perinuclear staining pattern of alcohol-fixed neutrophils, which is actually an artifact of the fixation process. ANCA specificity is determined by enzyme-linked immunosorbent assay (ELISA), with cANCA most commonly an antibody directed against PR3 and pANCA most commonly an antibody directed against MPO.
Nonspecific pANCA can occur in association with other autoimmune or inflammatory diseases, but they do not have the MPO specificity. The most common occurrence is in systemic lupus erythematosus. Other associated diseases include inflammatory bowel disease, sclerosing cholangitis, autoimmune hepatitis, rheumatoid arthritis, and Felty syndrome.
The ANCA-associated diseases are closely related and are distinguished by only a few clinical and pathologic criteria.
Wegener granulomatosis
WG is characterized by the presence of upper airway lesions, pulmonary infiltrates, and RPGN. Patients often present with pulmonary hemorrhage and renal failure. Pathologically, the lungs (and sometimes the upper airway lesions) show granulomatous inflammation. Of patients with WG, 80-90% have findings positive for ANCA and almost all have a cANCA (anti-PR3). A test result negative for ANCA does not exclude the presence of WG.
Churg-Strauss disease
This condition is characterized by allergic asthma and eosinophilia. Of patients with Churg-Strauss disease, 70-90% are positive for ANCA, primarily pANCAs.
Microscopic polyangiitis
MPA is characterized by pulmonary infiltrates and RPGN, often coupled with musculoskeletal system abnormalities or with neuropathy or central nervous system abnormalities. The term polyangiitis is used in preference to arteritis because vessels other than arteries are normally involved in the disease. Of patients with MPA, 80-90% have positive findings for ANCA and almost all have a pANCA (anti-MPO). A negative test result for ANCA does not exclude the presence of MPA. Isolated NCGN is the renal-limited form of MPA. Frequency:
- In the US: The exact frequency of ANCA-associated disease is unknown.
- Internationally: In the United Kingdom, the frequency is estimated at 2 cases per 100,000 persons. In Sweden, the frequency is estimated at 1 case per 100,000 persons.
Mortality/Morbidity: Massive pulmonary hemorrhage is the most common cause of death in patients presenting with ANCA-associated disease. However, once immunosuppressive therapy has begun, infection is more common.
Race: White persons are affected more frequently than African American persons. In the largest US study, the ratio was 7:1, with an overall regional population ratio of 3.5:1. However, African American persons were more likely to have a worse outcome. The reasons for this are not clear.
Sex: The male-to-female ratio in all studies is approximately 1:1.
Age: In most studies, the age range is 2-92 years. Peak incidence occurs in the middle of the sixth decade of life.
History: The most common prodrome of ANCA-associated vasculitis is flulike symptoms characterized by malaise, fever, arthralgias, myalgias, anorexia, and weight loss. This occurs in more than 90% of patients and can occur within days to months of the onset of nephritis or other manifestations of vasculitis. - Following the prodrome, the most common complaints are abdominal pain, painful cutaneous nodules or ulcerations, and a migratory polyarthropathy.
- When pulmonary or upper airway involvement is present, patients complain of sinusitis symptoms, cough, and hemoptysis.
Physical: Hypertension can be present but is not common. Unless specific findings are present, such as those listed below, the physical examination results are usually normal. Organs or systems affected by ANCA-associated disease are listed below. - Leukocytoclastic vasculitis is common (40-60%) and usually affects the lower extremities.
- Necrotizing arteritis can result in painful erythematous nodules, focal necrosis, ulceration, and livedo reticularis.
- Patients with WG or Churg-Strauss syndrome can also have granulomatous cutaneous nodules.
- Nail fold infarcts can be present.
- Mononeuritis multiplex is the most common nervous system manifestation of ANCA-associated disease. This condition is caused by inflammation of the epineural arteries and arterioles, which results in ischemia of the nerve tissue.
- Central nervous system disease can result from involvement of meningeal vessels and manifest as generalized seizures.
- Nervous system involvement is present in 30% of patients with MPA and 70% of patients with Churg-Strauss disease.
- Pain and elevation in tissue enzyme levels can result from inflammation in the arteries of skeletal muscle.
- Musculoskeletal involvement is present in 60% of patients with ANCA-associated disease.
- Arthritis is a very common symptom. It is usually symmetrical and migratory and usually involves the small joints.
- Arthralgias are also common, but this is not considered a marker of active vasculitis.
- Arteritis can result in ischemic ulceration in the GI tract, causing pain and bleeding, which is usually occult.
- The most serious complications of GI ischemia are intussusception and pancreatitis.
- GI involvement occurs in 50% of patients with ANCA.
- The diagnostic biopsy finding is proliferative NCGN.
- If overt renal disease is not present upon presentation, then the most common finding is microscopic hematuria.
- The prevalence rate of renal disease is 90% for those with MPA, 80% for those with WG, and 45% for those with Churg-Strauss disease.
- Pulmonary manifestations range from fleeting focal infiltrates to hemorrhagic alveolar capillaritis resulting in massive pulmonary hemorrhage and hemoptysis. This is the most deadly complication of ANCA disease.
- The prevalence rate of pulmonary findings is 50% in those with MPA, 90% in those with WG, and 80% in those with Churg-Strauss disease.
- Upper respiratory manifestations include sinusitis, otitis media, ulcers in the nasal mucosa, and subglottic stenosis.
- Iritis, uveitis, and conjunctivitis are the most common ocular manifestations of ANCA.
- Involvement occurs in approximately 2% of patients with ANCA-associated disease.
Causes: The cause of ANCA-associated disease is unknown. A genetic predisposition may exist for the development of this disease. Patients with WG are more likely to have abnormal alpha1-antitrypsin phenotypes. Patients who have the Z phenotype are more likely to have aggressive disease. Multiple studies have demonstrated that ANCA-activated neutrophils attack vascular endothelial cells. Because 97% of patients have a flulike prodrome, a viral etiology is possible. However, to date, no evidence exists to support this postulate.
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DIFFERENTIALS
| Section 4 of 9 |
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Amyloidosis, AA (Inflammatory)
Antiphospholipid Syndrome
Churg-Strauss Syndrome
Cryoglobulinemia
Glomerulonephritis, Diffuse Proliferative
Glomerulonephritis, Membranoproliferative
Glomerulonephritis, Nonstreptococcal Associated With Infection
Glomerulonephritis, Poststreptococcal
Goodpasture Syndrome
Hypertension
Hypertension, Malignant
Light-Chain Deposition Disease
Microscopic Polyangiitis
Multiple Myeloma
Nephritis, Lupus
Polyarteritis Nodosa
Wegener Granulomatosis
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Lab Studies:
- The most important requirement in the diagnosis of ANCA-associated disease is a high index of suspicion. Rapid diagnosis is essential for organ preservation. Laboratory studies include the following:
- CBC count with differential: Results are most likely normal, but anemia may be present if the patient has renal failure or is bleeding from the GI tract. Eosinophilia of 13% or greater is suggestive of Churg-Strauss disease.
- Routine chemistry: The most common abnormality is an increased serum creatinine level. However, the level can be normal at presentation. Tissue enzyme (ie, lactate dehydrogenase, creatine kinase) levels may be elevated if the amount of inflammation is significant enough to result in myalgias.
- Urinalysis with microscopy: Proteinuria is almost always present but is rarely greater than 2-3 g in 24 hours. Microscopic hematuria is invariably present and may be the only clue to renal disease at presentation. The presence of red cell casts indicates glomerular inflammation and is a very helpful clue.
- Erythrocyte sedimentation rate: Although a nonspecific finding, the rate is usually elevated with active disease.
- C-reactive protein: Levels are elevated and correspond with disease activity.
- Antinuclear antibody (ANA) titer: The ANA titer result is not positive in patients with ANCA-associated disease. A high ANA titer should direct the diagnosis toward systemic lupus erythematosus.
- ANCA with ELISA subtyping: More than 80% of patients with MPA are ANCA-positive, and most of these demonstrate pANCA with MPO specificity. Of patients with WG, 90% are ANCA-positive and most have cANCA with PR3 specificity, especially in pulmonary involvement. However, ANCA type and specificity is not pathognomonic for each of these clinical syndromes because some patients with WG are pANCA-positive and some patients with MPA are cANCA-positive.
- Cryoglobulins: The symptoms of cryoglobulinemia are very similar to those of ANCA-related disease. However, in persons with ANCA-related diseases, the cryoglobulin titer result should be negative.
- Hepatitis profile: ANCA-associated disease is not associated with hepatitis. However, hepatitis B is associated with polyarteritis nodosa and hepatitis C is associated with mixed cryoglobulinemia.
- Urine and serum protein electrophoresis: Perform this in any middle-aged or elderly person presenting with RPGN to exclude the presence of light-chain disease or overt multiple myeloma as a cause of the clinical findings.
Histologic Findings: Renal biopsy specimens show a diffuse, proliferative, necrotizing glomerulonephritis with crescent formation.
Medical Care: Therapy for ANCA-associated disease consists of a combination of corticosteroids and cyclophosphamide. Treatment with steroids alone results in a 3-fold increase in the risk of relapse compared to combination therapy. The only predictor of renal survival is the serum creatinine value at the time of diagnosis. Therefore, a high index of suspicion is imperative in order to establish the diagnosis quickly and institute treatment as soon as possible. Renal failure requiring dialysis is not a contraindication to treatment. Many patients can be removed from dialysis for an extended period (18 mo to 2 y). - The regimen used by the Glomerular Disease Collaborative Network at the University of North Carolina at Chapel Hill (Falk, 1990; Nachman, 1996) is as follows:
- Administer methylprednisolone at 7 mg/kg/d intravenously (not to exceed 1 g) for 3 days, followed by oral prednisone at 1 mg/kg/d (not to exceed 80 mg) for 3 weeks, and then oral prednisone at 2 mg/kg every other day (not to exceed 120 mg) for 3 months. This dose is decreased by 25% every 4 weeks until the patient stops taking prednisone.
- Administer cyclophosphamide either intravenously or orally. Intravenous therapy is initially administered at a dose of 0.5 g/m2, and the oral dose is 2 mg/kg. Both are adjusted according to a 2-week leukocyte nadir count (goal 3000-4000/mL). The maximum intravenous dose is 1 g/m2. Oral and intravenous cyclophosphamide appear to be equally efficacious. However, this remains an area of controversy, particularly in the case of WG, for which some advocate oral therapy. The advantage to using the intravenous preparation is that the risk of cumulative toxicity is lower because a lower total dose is used.
- Another protocol, which has been used widely and with success in Europe, is the substitution of azathioprine for cyclophosphamide after a 3-month induction period. Azathioprine is administered at 2 mg/kg orally in a single daily dose. This is continued for 6-12 months.
- Plasmapheresis may be a beneficial addition to therapy for patients who present with renal failure or those who progress despite treatment.
- Other medications have been used in an attempt to attain a remission, such as intravenous immunoglobulin, antithymocyte antibody, and humanized monoclonal antibody to CD4 and CD25. None of these therapies has been well studied. They appear in the literature as case reports.
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MEDICATION
| Section 7 of 9 |
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The goals of pharmacotherapy are to induce remission, to reduce morbidity, and to prevent complications.
Drug Category: Antineoplastic agents -- Has potent immunosuppressive properties. Drug Name
| Cyclophosphamide (Cytoxan) -- Chemically related to nitrogen mustards. Transformed primarily in the liver to active alkylating metabolites. The mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. PO and IV administration appear to be equally efficacious, although controversy exists. |
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| Adult Dose | 0.5 g/m2 IV; not to exceed 1 g/m2; alternatively, 2 mg/kg PO
Adjust both according to a 2-wk leukocyte nadir count (goal 3000-4000/mL)| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; severely depressed bone marrow function; preexisting malignancy |
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| Interactions | Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity |
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| Pregnancy |
D - Unsafe in pregnancy
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| Precautions | Risks are greater with PO route because larger total dose is administered; regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; increased risk of neoplasia, in particular transitional cell carcinoma of the bladder; also associated with lymphoma, sterility, and amenorrhea, which usually resolves after cessation of therapy |
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Drug Category: Corticosteroids -- Used for immunosuppressive and anti-inflammatory effects.Drug Name
| Methylprednisolone (Solu-Medrol) -- Decreases inflammation by suppressing migration of PMN leukocytes and reversing increased capillary permeability. After 3 d, switch to PO prednisone. |
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| Adult Dose | 7 mg/kg IV qd for 3 d; not to exceed 1 g/d |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; viral, fungal, or tubercular skin infections |
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| Interactions | Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels; phenobarbital, phenytoin, and rifampin may decrease levels (adjust dose); monitor patients for hypokalemia when taking concurrently with diuretics |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use |
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Drug Name
| Prednisone (Deltasone, Sterapred, Orasone) -- Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. |
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| Adult Dose | 1 mg/kg PO qd for 3 wk (not to exceed 80 mg/d), followed by 2 mg/kg PO qod for 3 mo (not to exceed 120 mg/d), and then decrease dose by 25% q4wk until patient stops taking prednisone |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy |
B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
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Drug Category: Immunosuppressant agents -- May be an effective substitution for cyclophosphamide.Drug Name
| Azathioprine (Imuran) -- Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. Protocol widely and successfully used in Europe is substitution of azathioprine for cyclophosphamide after 3-mo induction period. |
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| Adult Dose | 2 mg/kg PO qd for 6-12 mo; this follows a 3-mo induction period with cyclophosphamide |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; low levels of serum thiopurine methyl transferase |
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| Interactions | Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine |
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| Pregnancy |
D - Unsafe in pregnancy
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| Precautions | Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check thiopurine methyl transferase level prior to therapy and follow liver, renal, and hematologic function; pancreatitis rarely associated |
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Prognosis:
- Close follow-up care is extremely important in any patient with active vasculitis. The therapies for ANCA-associated vasculitides are not proven in large, randomized, controlled trials but are the standard of care according to consensus. The same can be said for the definitions of relapse, response, and treatment failure. The following criteria were established by Nachman et al before they performed a randomized control trial comparing immunosuppression regimens in patients diagnosed with MPA or isolated pauci-immune RPGN only (patients with WG were excluded).
- Remission
- Stabilization or improvement of renal function (as measured by serum creatinine value), resolution of hematuria, and resolution of extrarenal manifestations of systemic vasculitis
- Persistence of proteinuria not considered indicative of persistent disease activity
- Remission by therapy - Achievement of remission while still receiving immunosuppressive medication or corticosteroids (prednisone dose or equivalent at >7.5 mg/d)
- Treatment resistance
- Progressive decline in renal function with the persistence of an active urine sediment
- Persistence or new appearance of any extrarenal manifestation of vasculitis despite immunosuppressive therapy
- Relapse (at least one of the following)
- Rapid rise in serum creatinine concentration, accompanied by an active urine sediment
- Renal biopsy findings demonstrating active necrosis or crescent formation
- Hemoptysis, pulmonary hemorrhage, or new or expanding nodules without evidence for infection
- Active vasculitis of the respiratory or GI tracts as demonstrated by findings from endoscopic biopsy
- Iritis or uveitis
- New mononeuritis multiplex
- Necrotizing vasculitis identified based on findings from biopsy specimen of any tissue
Patient Education:
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BIBLIOGRAPHY
| Section 9 of 9 |
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Andrassy K, Kuster S, Waldherr R, Ritz E: Rapidly progressive glomerulonephritis: analysis of prevalence and clinical course. Nephron 1991; 59(2): 206-12[Medline].
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Bacani RA, Velasquez F, Kanter A, et al: Rapidly progressive (nonstreptococcal) glomerulonephritis. Ann Intern Med 1968 Sep; 69(3): 463-85[Medline].
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Couser WG: Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis 1988 Jun; 11(6): 449-64[Medline].
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Davies DJ, Moran JE, Niall JF, Ryan GB: Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed) 1982 Aug 28; 285(6342): 606[Medline].
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Falk RJ, Hogan S, Carey TS, Jennette JC: Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med 1990 Nov 1; 113(9): 656-63[Medline].
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Hall JB, Wadham BM, Wood CJ, et al: Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med 1984 Jun; 14(3): 277-8[Medline].
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Hogan SL, Nachman PH, Wilkman AS, et al: Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996 Jan; 7(1): 23-32[Medline].
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Jayne DR, Gaskin G, Pusey CD, Lockwood CM: ANCA and predicting relapse in systemic vasculitis. QJM 1995 Feb; 88(2): 127-33[Medline].
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Jennette JC: Renal Involvement in Systemic Vasculilits. In: Jennette JC, Olson JL, Schwartz MM, Silva FG, eds. Hepinstall's Pathology of the Kidney. 5th ed. Philadelphia, Pa: Lippincott-Raven; 1998: 1059-94.
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Nachman PH, Hogan SL, Jennette JC, Falk RJ: Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996 Jan; 7(1): 33-9[Medline].
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Pusey CD, Rees AJ, Evans DJ, et al: Plasma exchange in focal necrotizing glomerulonephritis without anti- GBM antibodies. Kidney Int 1991 Oct; 40(4): 757-63[Medline].
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Savige J, Davies D, Falk RJ, et al: Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int 2000 Mar; 57(3): 846-62[Medline].
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Stilmant MM, Bolton WK, Sturgill BC, et al: Crescentic glomerulonephritis without immune deposits: clinicopathologic features. Kidney Int 1979 Feb; 15(2): 184-95[Medline].
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