Rare diseases · Sign or symptom
Proteinuria
High urine protein levels
HP:0000093
What it means
Increased levels of protein in the urine.
Rare diseases that can present with this119
Very common80–99%
31- AA amyloidosis
- Alport syndrome-intellectual disability-midface hypoplasia-elliptocytosis syndrome
- Anti-glomerular basement membrane disease
- Atypical hemolytic uremic syndrome
- Autosomal recessive Alport syndrome
- Blepharophimosis-intellectual disability syndrome, Ohdo type
- Congenital nephrotic syndrome, Finnish type
- Cystinosis
- Dent disease
- Denys-Drash syndrome
- Donnai-Barrow syndrome
- Fibronectin glomerulopathy
- Frasier syndrome
- Galloway-Mowat syndrome
- Hereditary amyloidosis with primary renal involvement
- Idiopathic non-lupus full-house nephropathy
- Idiopathic steroid-resistant nephrotic syndrome
- Kawasaki disease
- Malakoplakia
- Mucopolysaccharidosis-like syndrome with congenital heart defects and hematopoietic disorders
- Multicentric carpo-tarsal osteolysis with or without nephropathy
- Nail-patella-like renal disease
- Oculocerebrorenal syndrome of Lowe
- Oligomeganephronia
- Pauci-immune glomerulonephritis
- Preeclampsia
- Schimke immuno-osseous dysplasia
- Severe oculo-renal-cerebellar syndrome
- Spastic paraplegia-nephritis-deafness syndrome
- Systemic lupus erythematosus
- X-linked Alport syndrome
Common30–79%
42- Adenine phosphoribosyltransferase deficiency
- AL amyloidosis
- ALECT2 amyloidosis
- Alport syndrome
- Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome
- Autosomal dominant Alport syndrome
- C3 glomerulopathy
- Cockayne syndrome type 1
- Congenital hyperinsulinism due to HNF4A deficiency
- Cryoglobulinemic vasculitis
- Distal 16p11.2 microdeletion syndrome
- Distal limb deficiencies-micrognathia syndrome
- Fabry disease
- Familial Mediterranean fever
- Granulomatosis with polyangiitis
- HELLP syndrome
- Heme oxygenase-1 deficiency
- Hemorrhagic fever-renal syndrome
- Hereditary pheochromocytoma-paraganglioma
- Hyperprolinemia type 1
- Hypocomplementemic urticarial vasculitis
- IgG4-related kidney disease
- Imerslund-Gräsbeck syndrome
- Immune-mediated thrombotic thrombocytopenic purpura
- Immunoglobulin-mediated membranoproliferative glomerulonephritis
- Insulin-resistance syndrome type B
- Juvenile nephropathic cystinosis
- LCAT deficiency
- Lysinuric protein intolerance
- MYH9-related syndromic thrombocytopenia
- Nephropathy-deafness-hyperparathyroidism syndrome
- Non-progressive predominantly posterior cavitating leukoencephalopathy with peripheral neuropathy
- Paternal uniparental disomy of chromosome 1 syndrome
- Pediatric systemic lupus erythematosus
- Primary membranoproliferative glomerulonephritis
- Renal hypoplasia, bilateral
- Renal nutcracker syndrome
- Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
- Severe growth deficiency-strabismus-extensive dermal melanocytosis-intellectual disability syndrome
- Sporadic pheochromocytoma/secreting paraganglioma
- Williams syndrome
- X-linked Alport syndrome-diffuse leiomyomatosis
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Protein in urine
Tracking symptoms like this for someone? Eleplan keeps symptoms, diagnoses, medications and every specialist in one plan.
Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.