Rare diseases that primarily involve the renal & kidney system. Each links to its full hub — overview, specialists, clinical trials, medications and community.
How this grouping is built. These conditions are grouped by a clinical keyword match on the disease name — the same conservative classifier Tomeko uses to pick each hub’s system motif. It is a Tomeko working grouping, not an official Orphanet organ classification, and some conditions affect more than one system. Every condition is itself catalogued from NIH GARD (Genetic and Rare Diseases Information Center) and Orphanet (orpha.net), and each hub cites its own sources. Sorted A→Z by name · 385 conditions · last built 2026-08-30.
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A55
- AApoAI Amyloidosis
- Acquired Cystic Disease-Associated Renal Cell Carcinoma
- Acquired Distal Renal Tubular Acidosis
- Acro-Renal-Mandibular Syndrome
- Acropectororenal Dysplasia
- Acrorenal Field Defect, Ectodermal Dysplasia, And Lipoatrophic Diabetes
- Acrorenal Syndrome
- Acrorenal Syndrome, Autosomal Recessive
- ACTH-Independent Adrenal Cushing Syndrome, Somatic
- ACTH-Independent Cushing Syndrome
- ACTH-Independent Macronodular Adrenal Hyperplasia 1
- ACTH-Independent Macronodular Adrenal Hyperplasia 2
- ACTH-Independent Macronodular Adrenal Hyperplasia 3
- Acute Adrenal Insufficiency
- Acute Diffuse Glomerulonephritis
- Acute Poststreptococcal Glomerulonephritis
- Acute Proliferative Glomerulonephritis
- Adrenal Carcinoma
- Adrenal Cortex Carcinoma
- Adrenal Cortex Neoplasm
- Adrenal Gland Cancer
- Adrenal Gland Ganglioneuroblastoma
- Adrenal Gland Myelolipoma
- Adrenal Gland Neuroblastoma
- Adrenal Hypoplasia, Cytomegalic Type
- Adrenal Medulla Cancer
- Adrenal Medulla Carcinoma
- Adrenal Medullary Hyperplasia
- Adrenal Pheochromocytoma
- Adult Familial Nephronophthisis-Spastic Quadriparesia Syndrome
- AFib Amyloidosis
- Aldosterone-Producing Adrenal Cortex Adenoma
- ALG9-Associated Autosomal Dominant Polycystic Kidney Disease
- Alport Syndrome
- Alport Syndrome 3b, Autosomal Recessive
- ALys Amyloidosis
- Amelogenesis Imperfecta Type 1G
- Aniridia-Renal Agenesis-Psychomotor Retardation Syndrome
- Anti-Basement Membrane Glomerulonephritis
- Apolipoprotein A-II Amyloidosis
- Arthrogryposis With Renal Dysfunction And Cholestasis Syndrome
- Arthrogryposis, Renal Dysfunction, And Cholestasis 1
- Arthrogryposis, Renal Dysfunction, And Cholestasis 2
- Autoimmune Glomerulonephritis
- Autoimmune Primary Adrenal Insufficiency
- Autosomal Dominant Alport Syndrome
- Autosomal Dominant Distal Renal Tubular Acidosis
- Autosomal Dominant Familial Hematuria-Retinal Arteriolar Tortuosity-Contractures Syndrome
- Autosomal Dominant Medullary Cystic Kidney Disease With Or Without Hyperuricemia
- Autosomal Dominant Polycystic Kidney Disease Type 1 With Tuberous Sclerosis
- Autosomal Dominant Proximal Renal Tubular Acidosis
- Autosomal Dominant Pseudohypoaldosteronism Type 1
- Autosomal Recessive Alport Syndrome
- Autosomal Recessive Distal Renal Tubular Acidosis
- Autosomal Recessive Polycystic Kidney Disease
B15
- Balkan Nephropathy
- Bartter Syndrome
- Benign Adrenal Gland Pheochromocytoma
- Benign Metanephric Tumor
- Benign Neoplasm Of Adrenal Gland
- Benign Neoplasm Of Adrenal Medulla
- Bilateral Massive Adrenal Hemorrhage
- Bilateral Multicystic Dysplastic Kidney
- Bilateral Renal Agenesis
- Bilateral Renal Dysplasia
- Bilateral Renal Hypoplasia
- BK-Virus Nephropathy
- BNAR Syndrome
- Branchiootorenal Syndrome 1
- Branchiootorenal Syndrome 2
C36
- C1q Nephropathy
- C3 Glomerulonephritis
- Castleman-Kojima Disease
- Cellular Congenital Mesoblastic Nephroma
- Central Nervous System Calcification-Deafness-Tubular Acidosis-Anemia Syndrome
- Childhood Adrenal Cortical Carcinoma
- Childhood Adrenal Gland Pheochromocytoma
- Childhood ALK-Rearranged Renal Cell Carcinoma
- Childhood Eosinophilic Solid And Cystic Renal Cell Carcinoma
- Childhood Renal Cell Carcinoma With MiT Translocations
- Chromophobe Renal Cell Carcinoma
- Chronic Rapidly Progressive Glomerulonephritis
- Classic Congenital Adrenal Hyperplasia
- Classic Congenital Adrenal Hyperplasia Due To 21-Hydroxylase Deficiency
- Classic Congenital Adrenal Hyperplasia Due To 21-Hydroxylase Deficiency, Salt Wasting Form
- Classic Congenital Adrenal Hyperplasia Due To 21-Hydroxylase Deficiency, Simple Virilizing Form
- Classic Congenital Lipoid Adrenal Hyperplasia Due To STAR Deficency
- Classic Congenital Mesoblastic Nephroma
- Classic Variant Of Chromophobe Renal Cell Carcinoma
- Clear Cell Carcinoma Of Kidney
- Clear Cell Papillary Renal Cell Carcinoma
- Complement 3 Glomerulopathy
- Congenital Adrenal Hyperplasia
- Congenital Adrenal Hyperplasia Due To Cytochrome P450 Oxidoreductase Deficiency
- Congenital Adrenal Hypoplasia, X-Linked
- Congenital Adrenal Insufficiency With 46, XY Sex Reversal OR 46,XY Disorder Of Sex Development-Adrenal Insufficiency Due To CYP11A1 Deficiency
- Congenital Lipoid Adrenal Hyperplasia Due To STAR Deficency
- Congenital Membranous Nephropathy Due To Maternal Anti-Neutral Endopeptidase Alloimmunization
- Congenital Mesoblastic Nephroma
- Congenital Nephrotic Syndrome
- Congenital Neutropenia-Myelofibrosis-Nephromegaly Syndrome
- Congenital Renal Artery Stenosis
- Cortisol-Producing Adrenal Cortex Adenoma
- Crescentic Glomerulonephritis
- Cushing Syndrome Due To Macronodular Adrenal Hyperplasia
- Cystic Partially Differentiated Nephroblastoma
D10
- Dent Disease
- Diabetes Insipidus, Nephrogenic, Autosomal
- Diabetes Insipidus, Nephrogenic, X-Linked
- Diffuse Glomerulonephritis
- Digenic Alport Syndrome
- Distal Renal Tubular Acidosis
- Donnai-Barrow Syndrome
- Double Uterus-Hemivagina-Renal Agenesis Syndrome
- Drug-Related Renal Tubular Dysgenesis
- Dyschondrosteosis-Nephritis Syndrome
E7
F16
- Faciocardiorenal Syndrome
- Familial Adrenal Hypoplasia With Absent Pituitary Luteinizing Hormone
- Familial Amyloid Nephropathy With Urticaria AND Deafness
- Familial Cystic Renal Disease
- Familial Idiopathic Steroid-Resistant Nephrotic Syndrome
- Familial Juvenile Hyperuricemic Nephropathy Type 1
- Familial Juvenile Hyperuricemic Nephropathy Type 2
- Familial Papillary Thyroid Carcinoma With Renal Papillary Neoplasia
- Familial Primary Hypomagnesemia With Hypercalciuria And Nephrocalcinosis
- Familial Renal Glucosuria
- Familial Renal Hypouricemia
- Familial Steroid-Resistant Nephrotic Syndrome With Sensorineural Deafness
- Familial Visceral Amyloidosis, Ostertag Type
- Fibulo-Ulnar Hypoplasia-Renal Anomalies Syndrome
- Finnish Congenital Nephrotic Syndrome
- Focal Embolic Glomerulonephritis
G2
H20
- Hantavirus Hemorrhagic Fever With Renal Syndrome
- Hantavirus Hemorrhagic Fever With Renal Syndrome, Dobrava-Belgrade Virus Type
- Hantavirus Hemorrhagic Fever With Renal Syndrome, Puumala Virus Type
- Hantavirus Hemorrhagic Fever With Renal Syndrome, Seoul Virus Type
- Hemorrhagic Fever-Renal Syndrome
- Hereditary Clear Cell Renal Cell Carcinoma
- Hereditary Leiomyomatosis And Renal Cell Cancer
- Hereditary Papillary Renal Cell Carcinoma
- Hereditary Renal Cell Carcinoma
- HIV-Associated Nephropathy
- Hydrocephalus-Blue Sclerae-Nephropathy Syndrome
- Hyperinsulinemic Hypoglycemia With Polycystic Kidney Disease
- Hyperkalemic Renal Tubular Acidosis
- Hypoparathyroidism, Deafness, Renal Disease Syndrome
- Hypophosphatemic Nephrolithiasis/osteoporosis 1
- Hypophosphatemic Nephrolithiasis/osteoporosis 2
- Hypotrichosis-Lymphedema-Telangiectasia-Renal Defect Syndrome
- Hypouricemia, Familial Renal, Due To Tubular Hypersecretion
- Hypouricemia, Renal
- Hypouricemia, Renal, 2
I21
- Idiopathic Membranous Glomerulonephritis
- Idiopathic Multidrug-Resistant Nephrotic Syndrome
- Idiopathic Nephrotic Syndrome
- Idiopathic Non-Lupus Full-House Nephropathy
- Idiopathic Steroid-Resistant Nephrotic Syndrome With Sensitivity To Second-Line Immunosuppressive Therapy
- Idiopathic Steroid-Sensitive Nephrotic Syndrome
- Idiopathic Steroid-Sensitive Nephrotic Syndrome With Secondary Steroid Resistance
- IgA Glomerulonephritis
- IMAGe Syndrome
- Immune-Complex Glomerulonephritis
- Immunoglobulin-Mediated Membranoproliferative Glomerulonephritis
- Immunotactoid Glomerulopathy
- Immunotactoid Or Fibrillary Glomerulopathy
- Infantile Nephronophthisis
- Infantile Nephropathic Cystinosis
- Infiltrating Renal Pelvis Transitional Cell Carcinoma
- Inherited Distal Renal Tubular Acidosis
- Inherited Isolated Adrenal Insufficiency Due To Partial CYP11A1 Deficiency
- Inherited Renal Tubular Disease
- Intrauterine Growth Retardation, Metaphyseal Dysplasia, Adrenal Hypoplasia Congenita, Genital Anomalies, And Immunodeficiency
- Isolated Adrenal Insufficiency
J4
K2
L6
M21
- Macrothrombocytopenia And Granulocyte Inclusions With Or Without Nephritis Or Sensorineural Hearing Loss
- Malignant Adrenal Gland Pheochromocytoma
- Malignant Tumor Of Adrenal Cortex
- Mayer-Rokitansky-Küster-Hauser Syndrome Type 2
- Melnick-Fraser Syndrome
- Membranoproliferative Glomerulonephritis, X-Linked
- Membranous Nephropathy
- Mesangial Proliferative Glomerulonephritis
- Mesangiocapillary Glomerulonephritis
- Mesangiocapillary Glomerulonephritis, Type II
- Metastatic Carcinoma In The Adrenal Medulla
- Midface Hypoplasia, Hearing Impairment, Elliptocytosis, And Nephrocalcinosis
- MIRAGE Syndrome
- MIT Family Translocation Renal Cell Carcinoma
- Mitochondrial DNA Depletion Syndrome, Hepatocerebrorenal Form
- Mucinous Tubular And Spindle Renal Cell Carcinoma
- Multicentric Carpo-Tarsal Osteolysis With Or Without Nephropathy
- Multicystic Kidney Dysplasia
- Multilocular Clear Cell Renal Cell Carcinoma
- Multiloculated Renal Cyst
- Multinodular Goiter-Cystic Kidney-Polydactyly Syndrome
N64
- Nail-Patella-Like Renal Disease
- Neonatal Renal Venous Thrombosis
- Neoplasm Of The Adrenal Gland
- Neoplasm Of The Adrenal Medulla
- Nephroblastoma
- Nephrocalcinosis
- Nephrogenic Diabetes Insipidus
- Nephrogenic Diabetes Insipidus-Intracranial Calcification Syndrome
- Nephrogenic Syndrome Of Inappropriate Antidiuresis
- Nephrogenic Systemic Fibrosis
- Nephrolithiasis/osteoporosis, Hypophosphatemic
- Nephronophthisis
- Nephronophthisis 1
- Nephronophthisis 11
- Nephronophthisis 12
- Nephronophthisis 13
- Nephronophthisis 14
- Nephronophthisis 15
- Nephronophthisis 16
- Nephronophthisis 18
- Nephronophthisis 19
- Nephronophthisis 20
- Nephronophthisis 3
- Nephronophthisis 4
- Nephronophthisis 7
- Nephronophthisis 9
- Nephronophthisis-Like Nephropathy 1
- Nephronophthisis-Like Nephropathy 2
- Nephropathic Cystinosis
- Nephropathy - Deafness - Hyperparathyroidism Syndrome
- Nephropathy, Progressive Tubulointerstitial, With Cholestatic Liver Disease
- Nephrosis-Deafness-Urinary Tract-Digital Malformations Syndrome
- Nephrotic Syndrome
- Nephrotic Syndrome 15
- Nephrotic Syndrome 16
- Nephrotic Syndrome Ocular Anomalies
- Nephrotic Syndrome Of Childhood - Steroid Sensitive
- Nephrotic Syndrome, IIa 26
- Nephrotic Syndrome, Type 10
- Nephrotic Syndrome, Type 11
- Nephrotic Syndrome, Type 12
- Nephrotic Syndrome, Type 13
- Nephrotic Syndrome, Type 17
- Nephrotic Syndrome, Type 18
- Nephrotic Syndrome, Type 19
- Nephrotic Syndrome, Type 2
- Nephrotic Syndrome, Type 20
- Nephrotic Syndrome, Type 21
- Nephrotic Syndrome, Type 22
- Nephrotic Syndrome, Type 23
- Nephrotic Syndrome, Type 24
- Nephrotic Syndrome, Type 3
- Nephrotic Syndrome, Type 4
- Nephrotic Syndrome, Type 6
- Nephrotic Syndrome, Type 8
- Nephrotic Syndrome, Type 9
- Neuroaxonal Dystrophy Renal Tubular Acidosis
- Neurofaciodigitorenal Syndrome
- Non-Amyloid Fibrillary Glomerulopathy
- Non-Classic Congenital Adrenal Hyperplasia
- Non-Classic Congenital Lipoid Adrenal Hyperplasia Due To STAR Deficency
- Non-Functioning Adrenal Cortex Adenoma
- Nonpapillary Renal Cell Carcinoma
- NPHP3-Related Meckel-Like Syndrome
O3
P27
- Papillary Renal Cell Carcinoma
- Pauci-Immune Glomerulonephritis
- Pauci-Immune Glomerulonephritis With ANCA
- Pauci-Immune Glomerulonephritis Without ANCA
- Pelvis Syndrome
- Perlman Syndrome
- Pierson Syndrome
- Polycystic Kidney Disease 2
- Polycystic Kidney Disease 3 With Or Without Polycystic Liver Disease
- Polycystic Kidney Disease 4
- Polycystic Kidney Disease 5
- Polycystic Kidney Disease 6 With Or Without Polycystic Liver Disease
- Polycystic Kidney Disease 7
- Polycystic Kidney Disease 8
- Polycystic Kidney Disease, Adult Type
- Postural Orthostatic Tachycardia Syndrome
- Primary Adrenal Insufficiency
- Primary Fanconi Syndrome
- Primary Hypomagnesemia
- Primary Immunodeficiency With Natural-Killer Cell Deficiency And Adrenal Insufficiency
- Primary Membranoproliferative Glomerulonephritis
- Primary Unilateral Adrenal Hyperplasia
- Progressive Hereditary Glomerulonephritis Without Deafness
- Proliferative Glomerulonephritis
- Proteinuria, Low Molecular Weight, With Hypercalciuria And Nephrocalcinosis
- Proximal Renal Tubular Acidosis
- Psychomotor Regression-Oculomotor Apraxia-Movement Disorder-Nephropathy Syndrome
R46
- Radio-Renal Syndrome
- Rapidly Progressive Glomerulonephritis
- Renal Agenesis
- Renal Aminoaciduria
- Renal Caliceal Diverticuli-Deafness Syndrome
- Renal Carnitine Transport Defect
- Renal Cell Adenocarcinoma
- Renal Cell Carcinoma
- Renal Cell Carcinoma Associated With Neuroblastoma
- Renal Cell Carcinoma Associated With Xp11.2 translocations/TFE3 Gene Fusions
- Renal Cell Carcinoma, Xp11-Associated
- Renal Coloboma Syndrome
- Renal Cysts And Diabetes Syndrome
- Renal Dysplasia
- Renal Dysplasia And Retinal Aplasia
- Renal Hypodysplasia/aplasia 1
- Renal Hypodysplasia/aplasia 2
- Renal Hypodysplasia/aplasia 3
- Renal Hypodysplasia/aplasia 4
- Renal Hypomagnesemia 2
- Renal Hypomagnesemia 4
- Renal Hypomagnesemia 5 With Ocular Involvement
- Renal Hypomagnesemia 6
- Renal Hypoplasia
- Renal Nutcracker Syndrome
- Renal Osteodystrophy
- Renal Pelvis Adenocarcinoma
- Renal Pelvis Papillary Urothelial Carcinoma
- Renal Pelvis Urothelial Carcinoma
- Renal Pelvis/ureter Urothelial Carcinoma
- Renal Sarcoma
- Renal Tuberculosis
- Renal Tubular Acidosis
- Renal Tubular Acidosis 3
- Renal Tubular Acidosis With Progressive Nerve Deafness
- Renal Tubular Acidosis, Distal, 3, With Or Without Sensorineural Hearing Loss
- Renal Tubular Acidosis, Distal, 4, With Hemolytic Anemia
- Renal Tubular Dysgenesis
- Renal Tubular Dysgenesis - ACE
- Renal Tubular Dysgenesis Due To Twin-Twin Transfusion
- Renal Tubular Dysgenesis Of Genetic Origin
- Renal-Genital-Middle Ear Anomalies
- Renal-Hepatic-Pancreatic Dysplasia
- Renal-Hepatic-Pancreatic Dysplasia 1
- Renal-Hepatic-Pancreatic Dysplasia 2
- RHYNS Syndrome
S11
- Sarcomatoid Renal Cell Carcinoma
- Schimke Immuno-Osseous Dysplasia
- Senior-Boichis Syndrome
- SERKAL Syndrome
- Sex Hormone-Producing Adrenal Cortex Adenoma
- Sphingosine Phosphate Lyase Insufficiency Syndrome
- Sporadic Idiopathic Steroid-Resistant Nephrotic Syndrome
- Steroid-Resistant Nephrotic Syndrome
- Structural Heart Defects And Renal Anomalies Syndrome
- Subacute Glomerulonephritis
- Syndactyly-Telecanthus-Anogenital And Renal Malformations Syndrome
T9
- TFEB-Rearranged Renal Cell Carcinoma
- Thymic-Renal-Anal-Lung Dysplasia
- Thyrocerebrorenal Syndrome
- Torticollis-Keloids-Cryptorchidism-Renal Dysplasia Syndrome
- Townes Syndrome
- Tryptophan Malabsorption Syndrome
- Tubulocystic Renal Cell Carcinoma
- Tubulointerstitial Nephritis And Uveitis Syndrome
- Tyrosinemia Type I