Différences entre versions de « Glomérulopathies »
De médecine.top
(27 versions intermédiaires par le même utilisateur non affichées) | |||
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| '''> 3 − 3,5 g/24h''' | | '''> 3 − 3,5 g/24h''' | ||
|- | |- | ||
− | | | + | | Sérum |
| | | | ||
− | | < 30 g/l | + | | Albuminémie < 30 g/l<br>Cholestérol sérique ↑<br>Hypercoagulabilité<br>Risque infectieux ↑ |
|- | |- | ||
| Fonction rénale | | Fonction rénale | ||
Ligne 32 : | Ligne 32 : | ||
| Sédiment urinaire | | Sédiment urinaire | ||
| colspan="2" | Erythrocytes dysmorphiques et/ou cylindres érythrocytaires | | colspan="2" | Erythrocytes dysmorphiques et/ou cylindres érythrocytaires | ||
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|} | |} | ||
== Syndrome néphritique == | == Syndrome néphritique == | ||
− | + | === Glomérulonéphrite rapidement progressive === | |
− | + | * <u>Caractéristiques:</u> | |
− | + | *# at least a 50% decline in glomerular filtration rate over a short period (usually days to weeks) with | |
− | + | *# pathology findings of extensive glomerular crescents | |
− | + | * <u>Entités associées:</u> | |
− | + | ** [[Vasculites]] ANCA positives (polyangéites microscopiques: [[Vasculites#Granulomatose_de_Wegener|Granulomatose de Wegener]], [[Vasculites#Polyang.C3.A9ite_microscopique|Polyangéite microscopique]]) | |
− | + | ** Syndrome de Goodpasture ([[Glomérulopathies#Glom.C3.A9rulon.C3.A9phrite_.C3.A0_anticorps_anti-membrane_basale|↓ anticorps anti-membrane basale glomérulaire]]) | |
− | + | ** Lupus érythémateux disséminé (LED) | |
− | + | ** [[Glomérulopathies#Néphropathie_à_IgA|↓ Néphropathie à IgA (rare)]] | |
− | + | ||
− | + | === Glomérulonéphrite à anticorps anti-membrane basale === | |
− | |||
− | * | ||
− | * Syndrome de Goodpasture (anticorps anti-membrane basale glomérulaire) | ||
− | * Lupus érythémateux disséminé (LED) | ||
− | * Néphropathie à IgA (rare) | ||
− | |||
* Lung involvement (Goodpasture syndrome) occurs in >50% of patients | * Lung involvement (Goodpasture syndrome) occurs in >50% of patients | ||
* Treatment for anti-GBM disease consists of plasmapheresis, pulse glucocorticoids (high doses of intravenous glucocorticoids over a short period of time) followed by oral prednisone, and cyclophosphamide. | * Treatment for anti-GBM disease consists of plasmapheresis, pulse glucocorticoids (high doses of intravenous glucocorticoids over a short period of time) followed by oral prednisone, and cyclophosphamide. | ||
− | + | === Néphropathie à IgA === | |
− | * Recurrent gross hematuria, in which macroscopic hematuria occurs in the setting of an upper respiratory infection (synpharyngitic hematuria) is a common presentation in younger patients | + | <u>Synonyme:</u> maladie de Berger |
− | * Antiproteinuric therapy using an ACE inhibitor or angiotensin receptor blocker (ARB) is the hallmark for treating IgA nephropathy and remains the most proven therapy in slowing progression of the disease. | + | * <u>Epidémiologie:</u> maladie glomérulaire la plus répandue au monde<ref>https://www.revmed.ch/revue-medicale-suisse/2024/revue-medicale-suisse-863/nephropathie-a-iga-le-debut-d-une-nouvelle-ere-therapeutique</ref> |
+ | * <u>Physiopathologie:</u> dépôts mésangiaux d'IgA aberrants qui entraînent une inflammation locale, aboutissant à des lésions glomérulaires constituées, responsables d’une fuite de protéines et d’hématies dans l’urine<ref>RevMed</u> | ||
+ | * <u>Clinique:</u> Recurrent gross hematuria, in which macroscopic hematuria occurs in the setting of an upper respiratory infection (synpharyngitic hematuria) is a common presentation in younger patients | ||
+ | * <u>Diagnostic:</u> '''biopsie''' | ||
+ | * <u>Traitement:</u> | ||
+ | ** Antiproteinuric therapy using an '''ACE inhibitor or angiotensin receptor blocker (ARB)''' is the hallmark for treating IgA nephropathy and remains the most proven therapy in slowing progression of the disease. | ||
+ | ** iSGLT-2, corticostéroïdes/budésonide, ... | ||
+ | * <u>Suivi:</u> | ||
+ | ** '''Protéinurie (cible <1g/24h)''', (suivi en % des érythrocytes glomérulaires) | ||
+ | |||
+ | === Glomérulonéphrite post-infectieuse === | ||
+ | * <u>Diagnostic:</u> '''Fraction C3 du complément abaissée dans 90% des cas''', biopsie rénale à discuter | ||
+ | * <u>Traitement:</u> cause sous-jacente, glucocorticoïdes | ||
+ | ==== Forme à début aigu curable ==== | ||
+ | ==== Forme à début aigu d'évolution chronique ==== | ||
+ | ==== Forme à début aigu avec insuffisance rénale rapidement progressive ==== | ||
− | + | === Glomérulonéphrite membrano-proliférative === | |
− | ==== | + | ==== Type 1: forme à complexes immuns ==== |
* An immune-complex MPGN, with or without cryoglobulinemia, is the classic form of kidney involvement seen in patients with hepatitis C virus infection | * An immune-complex MPGN, with or without cryoglobulinemia, is the classic form of kidney involvement seen in patients with hepatitis C virus infection | ||
* The more common pattern is immune-complex deposition with the presence of both immunoglobulin (IgG, IgM, and/or IgA) and complement (C1q and/or C3) on immunofluorescence, which infers that the classical pathway has been activated by an inciting cause or event that generally falls into one of three major categories: infectious, autoimmune, or malignancy associated. The most common is infectious, specifically infection with hepatitis C virus (HCV). | * The more common pattern is immune-complex deposition with the presence of both immunoglobulin (IgG, IgM, and/or IgA) and complement (C1q and/or C3) on immunofluorescence, which infers that the classical pathway has been activated by an inciting cause or event that generally falls into one of three major categories: infectious, autoimmune, or malignancy associated. The most common is infectious, specifically infection with hepatitis C virus (HCV). | ||
− | ==== | + | ==== Type 2: forme médiée par le complément ==== |
== Syndrome néphrotique == | == Syndrome néphrotique == | ||
− | + | === Hyalinose segmentaire et focale === | |
− | + | * Synonymes: '''glomérulosclérose focale et segmentaire (FSGS)''' | |
− | |||
− | |||
− | |||
− | |||
− | |||
− | |||
− | |||
− | Synonymes: glomérulosclérose focale et segmentaire (FSGS) | ||
* <u>Traitement non spécifique néphroprotecteur:</u> IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique | * <u>Traitement non spécifique néphroprotecteur:</u> IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique | ||
* <u>Traitement spécifique:</u> '''glucocorticoïdes''' si protéinurie néphrotique et si FSGS primaire suspecté | * <u>Traitement spécifique:</u> '''glucocorticoïdes''' si protéinurie néphrotique et si FSGS primaire suspecté | ||
** <u>Deuxième ligne:</u> calcineurin inhibitors, mycophenolate mofetil, alkylating agents, and rituximab si absence de réponse aux glucocorticoïdes | ** <u>Deuxième ligne:</u> calcineurin inhibitors, mycophenolate mofetil, alkylating agents, and rituximab si absence de réponse aux glucocorticoïdes | ||
− | + | === Glomérulonéphrite extra-membraneuse === | |
* Lésion principale: podocyte et membrane basale glomérulaire | * Lésion principale: podocyte et membrane basale glomérulaire | ||
* Mécanisme: auto-immun (complexes immuns et IgG in situ) | * Mécanisme: auto-immun (complexes immuns et IgG in situ) | ||
− | * Diagnostic: biopsie, '''dosage des anticorps | + | * <u>Diagnostic:</u> biopsie, '''dosage des anticorps anti-phospholipase A2 (PLA2)''' |
* <u>Traitement non spécifique néphroprotecteur:</u> IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique | * <u>Traitement non spécifique néphroprotecteur:</u> IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique | ||
− | * <u>Complications:</u> In a large retrospective cohort of clotting complications in membranous glomerulopathy, >70% of the clots occurred within 2 years of diagnosis, and the risk of clotting markedly increased once albumin levels dropped below 2.8 g/dL (28 g/L) (OR, 2.53; P = 0.02, compared with albumin ≥2.8 g/dL [28 g/L]). | + | * <u>Complications:</u> |
− | + | ** '''Thrombophilie (en particulier de la veine rénale)''' → In a large retrospective cohort of clotting complications in membranous glomerulopathy, >70% of the clots occurred within 2 years of diagnosis, and the risk of clotting markedly increased once albumin levels dropped below 2.8 g/dL (28 g/L) (OR, 2.53; P = 0.02, compared with albumin ≥2.8 g/dL [28 g/L]). | |
+ | ==== Forme primaire ==== | ||
* Patients with newly diagnosed primary forms of membranous glomerulopathy are usually observed for 6 to 12 months on conservative therapy (renin-angiotensin system blockade, cholesterol-lowering medication, and edema management) before initiating a course of immunosuppression for patients with persistent nephrotic-range proteinuria. The observation period allows patients a chance to achieve spontaneous remission, which occurs in approximately 30% within 1 to 2 years of diagnosis. | * Patients with newly diagnosed primary forms of membranous glomerulopathy are usually observed for 6 to 12 months on conservative therapy (renin-angiotensin system blockade, cholesterol-lowering medication, and edema management) before initiating a course of immunosuppression for patients with persistent nephrotic-range proteinuria. The observation period allows patients a chance to achieve spontaneous remission, which occurs in approximately 30% within 1 to 2 years of diagnosis. | ||
* <u>Traitement immunosuppresseur (3 lignes):</u> | * <u>Traitement immunosuppresseur (3 lignes):</u> | ||
Ligne 95 : | Ligne 93 : | ||
** Ciclosporine/tacrolimus | ** Ciclosporine/tacrolimus | ||
** Rituximab | ** Rituximab | ||
− | + | ==== Forme secondaire ==== | |
− | * associée au cancer (côlon, prostate), infections, maladies auto-immunes (lupus), médicaments (AINS, captopril) | + | * associée au cancer (côlon, prostate), infections ([[Hépatites virales|hépatites B et C]]), maladies auto-immunes (lupus), médicaments (AINS, captopril) |
* The initial step in the management of newly diagnosed membranous glomerulopathy is to evaluate for secondary forms of the disease, which account for approximately 25% of cases. | * The initial step in the management of newly diagnosed membranous glomerulopathy is to evaluate for secondary forms of the disease, which account for approximately 25% of cases. | ||
− | + | === Minimal Change Disease (MCD) === | |
− | + | ==== Primaire ==== | |
− | + | ==== Secondaire ==== | |
− | + | === Néphropathie diabétique === | |
* The hallmark clinical features include a proteinuric form of chronic kidney disease in a patient with long-standing diabetes mellitus and evidence of other (microvascular and/or macrovascular) complications of disease. | * The hallmark clinical features include a proteinuric form of chronic kidney disease in a patient with long-standing diabetes mellitus and evidence of other (microvascular and/or macrovascular) complications of disease. | ||
* cornerstone of treatment: improved glucose control, and blockade of the renin-angiotensin system (RAS) with an ACE inhibitor or ARB using the maximal tolerated dose. | * cornerstone of treatment: improved glucose control, and blockade of the renin-angiotensin system (RAS) with an ACE inhibitor or ARB using the maximal tolerated dose. | ||
== Indications à la biopsie == | == Indications à la biopsie == | ||
− | The decision to pursue a kidney biopsy should be individualized for each patient, but, in general, a kidney biopsy appears justified for patients with two or more of the following four findings: hematuria, proteinuria >1000 mg/24 h, reduced kidney function (glomerular filtration rate <60 mL/min/1.73 m2), and/or positive serologies for systemic diseases with known potential for kidney involvement (for example, hepatitis B or C virus infection, systemic lupus erythematosus, and ANCA seropositivity). | + | The decision to pursue a kidney biopsy should be individualized for each patient, but, in general, a kidney biopsy appears justified for patients with two or more of the following four findings: |
+ | * hematuria, | ||
+ | * proteinuria >1000 mg/24 h, | ||
+ | * reduced kidney function (glomerular filtration rate <60 mL/min/1.73 m2), | ||
+ | * and/or positive serologies for systemic diseases with known potential for kidney involvement (for example, hepatitis B or C virus infection, systemic lupus erythematosus, and ANCA seropositivity). | ||
kidney biopsy is not indicated in the setting of small echogenic kidneys <9 cm in size, which signifies chronic irreversible disease. | kidney biopsy is not indicated in the setting of small echogenic kidneys <9 cm in size, which signifies chronic irreversible disease. | ||
+ | |||
+ | == Références == | ||
+ | * '''2012:''' https://www.revmed.ch/RMS/2012/RMS-330/Proteinurie-rappel-physiologique-et-applications-pratiques | ||
[[Category:Néphrologie]] | [[Category:Néphrologie]] |
Version actuelle datée du 28 mars 2024 à 10:10
Terminologie
- Lésion diffuse (tous les glomérules atteints) vs focale (<50% des glomérules atteints)
- Lésion globale (l'entier du glomérule) vs segmentaire (<50% du glomérule)
- Lésion proliférative, sclérosante, nécrosante
Résumé
Syndrome néphritique | Syndrome néphrotique | |
---|---|---|
Physiopathologie | Inflammation diffuse des glomérules = glomérulonéphrite | ↑ perméabilité de la membrane basale glomérulaire |
Protéinurie | < 3 g/24h | > 3 − 3,5 g/24h |
Sérum | Albuminémie < 30 g/l Cholestérol sérique ↑ Hypercoagulabilité Risque infectieux ↑ | |
Fonction rénale | Hématurie (microscopique ou macroscopique) Insuffisance rénale avec oligurie (inconstante et de degré variable) |
Forme pure: pas d'insuffisance rénale organique |
Clinique (OMI, HTA) | Triade de Volhard: HTA, hématurie, oedèmes | OMI |
Sédiment urinaire | Erythrocytes dysmorphiques et/ou cylindres érythrocytaires |
Syndrome néphritique
Glomérulonéphrite rapidement progressive
- Caractéristiques:
- at least a 50% decline in glomerular filtration rate over a short period (usually days to weeks) with
- pathology findings of extensive glomerular crescents
- Entités associées:
- Vasculites ANCA positives (polyangéites microscopiques: Granulomatose de Wegener, Polyangéite microscopique)
- Syndrome de Goodpasture (↓ anticorps anti-membrane basale glomérulaire)
- Lupus érythémateux disséminé (LED)
- ↓ Néphropathie à IgA (rare)
Glomérulonéphrite à anticorps anti-membrane basale
- Lung involvement (Goodpasture syndrome) occurs in >50% of patients
- Treatment for anti-GBM disease consists of plasmapheresis, pulse glucocorticoids (high doses of intravenous glucocorticoids over a short period of time) followed by oral prednisone, and cyclophosphamide.
Néphropathie à IgA
Synonyme: maladie de Berger
- Epidémiologie: maladie glomérulaire la plus répandue au monde[1]
- Physiopathologie: dépôts mésangiaux d'IgA aberrants qui entraînent une inflammation locale, aboutissant à des lésions glomérulaires constituées, responsables d’une fuite de protéines et d’hématies dans l’urine<ref>RevMed
- Clinique: Recurrent gross hematuria, in which macroscopic hematuria occurs in the setting of an upper respiratory infection (synpharyngitic hematuria) is a common presentation in younger patients
- Diagnostic: biopsie
- Traitement:
- Antiproteinuric therapy using an ACE inhibitor or angiotensin receptor blocker (ARB) is the hallmark for treating IgA nephropathy and remains the most proven therapy in slowing progression of the disease.
- iSGLT-2, corticostéroïdes/budésonide, ...
- Suivi:
- Protéinurie (cible <1g/24h), (suivi en % des érythrocytes glomérulaires)
Glomérulonéphrite post-infectieuse
- Diagnostic: Fraction C3 du complément abaissée dans 90% des cas, biopsie rénale à discuter
- Traitement: cause sous-jacente, glucocorticoïdes
Forme à début aigu curable
Forme à début aigu d'évolution chronique
Forme à début aigu avec insuffisance rénale rapidement progressive
Glomérulonéphrite membrano-proliférative
Type 1: forme à complexes immuns
- An immune-complex MPGN, with or without cryoglobulinemia, is the classic form of kidney involvement seen in patients with hepatitis C virus infection
- The more common pattern is immune-complex deposition with the presence of both immunoglobulin (IgG, IgM, and/or IgA) and complement (C1q and/or C3) on immunofluorescence, which infers that the classical pathway has been activated by an inciting cause or event that generally falls into one of three major categories: infectious, autoimmune, or malignancy associated. The most common is infectious, specifically infection with hepatitis C virus (HCV).
Type 2: forme médiée par le complément
Syndrome néphrotique
Hyalinose segmentaire et focale
- Synonymes: glomérulosclérose focale et segmentaire (FSGS)
- Traitement non spécifique néphroprotecteur: IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique
- Traitement spécifique: glucocorticoïdes si protéinurie néphrotique et si FSGS primaire suspecté
- Deuxième ligne: calcineurin inhibitors, mycophenolate mofetil, alkylating agents, and rituximab si absence de réponse aux glucocorticoïdes
Glomérulonéphrite extra-membraneuse
- Lésion principale: podocyte et membrane basale glomérulaire
- Mécanisme: auto-immun (complexes immuns et IgG in situ)
- Diagnostic: biopsie, dosage des anticorps anti-phospholipase A2 (PLA2)
- Traitement non spécifique néphroprotecteur: IEC/ARA (protection de la fonction rénale), diurétique (contrôle de l'oedème) et hypolipémiant selon profil lipidique
- Complications:
- Thrombophilie (en particulier de la veine rénale) → In a large retrospective cohort of clotting complications in membranous glomerulopathy, >70% of the clots occurred within 2 years of diagnosis, and the risk of clotting markedly increased once albumin levels dropped below 2.8 g/dL (28 g/L) (OR, 2.53; P = 0.02, compared with albumin ≥2.8 g/dL [28 g/L]).
Forme primaire
- Patients with newly diagnosed primary forms of membranous glomerulopathy are usually observed for 6 to 12 months on conservative therapy (renin-angiotensin system blockade, cholesterol-lowering medication, and edema management) before initiating a course of immunosuppression for patients with persistent nephrotic-range proteinuria. The observation period allows patients a chance to achieve spontaneous remission, which occurs in approximately 30% within 1 to 2 years of diagnosis.
- Traitement immunosuppresseur (3 lignes):
- Glucocorticoïdes et agent alkylant (cyclophosphamide: Endoxan)
- Ciclosporine/tacrolimus
- Rituximab
Forme secondaire
- associée au cancer (côlon, prostate), infections (hépatites B et C), maladies auto-immunes (lupus), médicaments (AINS, captopril)
- The initial step in the management of newly diagnosed membranous glomerulopathy is to evaluate for secondary forms of the disease, which account for approximately 25% of cases.
Minimal Change Disease (MCD)
Primaire
Secondaire
Néphropathie diabétique
- The hallmark clinical features include a proteinuric form of chronic kidney disease in a patient with long-standing diabetes mellitus and evidence of other (microvascular and/or macrovascular) complications of disease.
- cornerstone of treatment: improved glucose control, and blockade of the renin-angiotensin system (RAS) with an ACE inhibitor or ARB using the maximal tolerated dose.
Indications à la biopsie
The decision to pursue a kidney biopsy should be individualized for each patient, but, in general, a kidney biopsy appears justified for patients with two or more of the following four findings:
- hematuria,
- proteinuria >1000 mg/24 h,
- reduced kidney function (glomerular filtration rate <60 mL/min/1.73 m2),
- and/or positive serologies for systemic diseases with known potential for kidney involvement (for example, hepatitis B or C virus infection, systemic lupus erythematosus, and ANCA seropositivity).
kidney biopsy is not indicated in the setting of small echogenic kidneys <9 cm in size, which signifies chronic irreversible disease.