Pathologie (Fach) / Niere (Lektion)

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• Polycystic kidney disease ADPKD: ∼1/1,000; one of the most common inherited kidney diseases in humansARPKD: ∼1/20,000 Autosomal dominant PKD:- Mutation on chromosome 16 (85% of cases) or 4 (15% of cases) with an alteration of polycystin-1 or 2- Manifests in adulthood (typically age > 30 years) - Cysts vary in size- Cysts in liver, seminal vesicles, pancreas, vasculature (aortic root dilation → heart failure; berry aneurysms → subarachnoid hemorrhages), diverticulosis, mitral valve prolapse Autosomal recessive PKD:- Mutation on chromosome 6, which codes fibrocystin protein- Manifests in infancy with renal failure before birth → oligohydromnios → Potter sequence (clubbed feet, flattened nose, pulmonary hypoplasia → respiratory insufficiency)- Equally sized cysts- Congenital hepatic fibrosis → portal hypertension → varices, upper GI bleeds, hemorrhoids, splenomegaly- Defects in bile ducts → dilation → cholestasis, ascending cholangitis Treatment: ACE inhibitors, AT-R blockers, dialysis/kidney transplant
• Polyzystische Niere 1. Zystisch erweiterte Hohlräume, die von tubulären Epithelzellen ausgekleidet sind 2. Zysten im Kortex und Medulla 3. Vereinzelte eingeschlossene Glomeruli 4. Atrophisches Nierengewebe, teils mit geringer Begleitentzündung 5. Zeichen alter Blutungen: Hämosiderin-tragende Makrophagen
• Multicystic dysplastic kidney Congenital disease where one or both kidneys don't form correctly because the ureteric bud fails to develop properly.- Most commonly unilateral. May be a result of abnormal induction of metanephric blastema by the ureteric bud. → Urine has nowhere to go → Fluid-filled cysts out of abnormal connective tissue and cartilage → renal failure. - Sporadic - Normal kidney might be able to preserve kidney function- If bilateral → dialysis/transplant, many die
• Medullary cystic kidney disease Autosomal dominant disorder that affects the renal tubules and interstitium.- Presents in adulthood Symptoms: Polyuria or polydipsia, family history, salt wasting Diagnosis: Renal biopsy and genetic testing Pathology:- Tubulointerstitial fibrosis and inflammation → Tubules lose ability to concentrate urine → Polyuria- Tubular atrophy- Cysts in the corticomedullary junction Treatment: Dialysis, transplant
• Renal cyst disorder - Autosomal dominant polycystic kidney disease - Autosomal recessive polycystic kidney disease - Medullary cystic kidney disease (= autosomal dominant tubulointerstitial kidney disease) - Multicystic dysplastic kidney (sporadic) - Simple and complex cysts (sporadic)
• Diabetic nephropathy #1 leading cause of end-stage renal disease. Non-enzymatic glycylation: - Efferent arteriole stiffening (hyaline arteriosclerosis) → Obstruction to blood flow → increased pressure → increase in glomerular filtration rate (1st stage)- Basement membrane thickening → filtration splits widen → more permeable for proteins - Mesangial cells produce more structural matrix → Kimmelstiel-Wilson nodules 1st stage: Hyperfiltration 2nd stage: Albuminuria3rd stage: Decreased GFR4th stage: End-stage renal disease Screening:- Microalbuminuria (30-300 mg of albumin/day) - Macroalbuminuria (>300 mg of albumin/day) Pathology:- Mesangial expansion- Glomerular basement membrane thickening- Glomerulosclerosis (later stages): may be diffuse (most common) or pathognomonic nodular glomerulosclerosis (Kimmelstiel-Wilson nodules)- Hyaline thickening of the arteriole wall Treatment: - Control  hypertension: ACE inhibitors, AT-R blockers- Control hyperglycemia
• Diabetische Glomerulosklerose 1. Hyaline Arteriolosklerose  2. Vermehrung der mesangialen Matrix = Kimmelstiel-Wilson Nodule 3. Verdickte Basalmembran 4. Tubulusatrophie mit interstitieller Fibrose 5. Begleitentzündung
• Niereninfarkt 1. Koagulationsnekrose: Verstärkte Zytoplasmaazidophilie und Verlust der Kernzeichnung bei erhaltener Architektur 2. Hämorrhagischer Randsaum 3. Leukozytärer Randsaum aus neutrophilen Granulozyten
• Amyloidosis Tissue damage from misfolded protein deposits. Amyloid bilds β-sheets that deposit in extracellular space of tissue and cause damage. Systemic amyloidosis:- AL amyloidosis: Light chain is misfolded and deposited. Seen in plasma cell disorders (eg, multiple myeloma). - AA amyloidosis: Serum amyloid A is an acute phase reactant that accumualtes in chronic inflammation. Seen in rheumatoid arthritis, inflammatory bowel diseases, cancers, familial mediterranean fever.- Dialysis-related amyloidosis (β2-microglobulin): Seen in patients with ESRD and/or on long-term dialysis- Kidney: Damages podocytes → proteinuria >3.5 g/d, hypoalbuminuria → edema, hyperlipidemia (= nephrotic syndrome)- Heart: Stiffens heart walls → restrictive cardiomyopathy → congestive heart failure; intereferes with electrical conductance → arrhythmia- Intestines: Affects tips of villi → diarrhea, malabsorption- Peripheral nerves → systemic and autonomic neuropathy- Enlargement of liver, spleen, tongue Localized amyloidosis:- Alzheimer's disease: Aβ-peptides form extracellular plaques and interfere with neuron signaling; cause amyloid angiopathy - Familial amyloid cardiomyopathy (ATTR amyloidosis): Genetic disorder with mutant TTR → restrictive cardiomyopathy- Senile amyloidosis: Normal TTR gradually deposited, seen in elderly- Diabetes mellitus type II: Islet amyloid peptide (amylin) aggregates between beta cells in islets of Langerhans Diagnosis:- Biopsy of involved organ / Fat pad biopsy (fat taken from abdomen)- Congo red stain → pink. Polarized light → apple green.
• Amyloidnephropathie 1. Homogene, eosinrote Ablagerungen in den Arterienwände 2. Homogene, eosinrote Ablagerungen in den Glomerulusschlingen. 2. Glomeruläre Struktur im Endstadium weitgehend zerstört  Es handelt sich bei der Nierenamyloidose meist um eine AL-Amyloidose (90%), seltener AA-Amyloidose. Beweisend ist die grüne Doppelbrechung in polarisiertem Licht.
• Hydronephrosis Condition where excessive amount of urine causes the kidney to dilate. Etiology:- Fetus: Congenital ureteropelvic junction obstruction, vesicoureteral reflux, ureterocele, posterior urethral valves- Kidney stones, external compression- Prostatic hyperplasia Nephron destruction → increased serum creatinine & electrolyte imbalancesDilated ureter and renal pelvisCompression atrophy = Thinning of the renal medulla and cortex Symptoms:- From obstruction: Flank or groin pain, urinary tract infections- From hydronephrosis: postrenal azotemia Diagnosis: - UltrasoundGrade 0 = No dilationGrade 1= Dilation of renal pelvis (not calyces)Grade 2 = Dilation of renal pelvis & calycesGrade 3 = Moderate dilation, mild cortical thinning, flattening of papillaeGrade 4 = Severe dilation and cortical thinning- Intravenous urography or pyelography often used for children- CT scan Treatment: Relieve obstruction- Acutely: Nephrostomy tube- Chronically: Ureteric stent, pyeloplasty (surgical remake of renal pelvis)- Lower obstructions: Urinary or suprapubic catheter
• Refluxnephropathie 1. Atrophie der Tubuli 2. Pseudostrumiforme Umwandlung 3. Interstitielle Entzündung 4. Arterien mit adaptativer Intimafibrose 5. Sklerosierung der Glomeruli 6. Ektatisch ausgeweitetes Nierenbecken Der Reflux führt charakteristischerweise zu einer polbetonten Läsion der Nieren.
• Membranous glomerulonephritis Immune complexes deposit in the glomerular basement membrane which becomes inflammed and damage → nephrotic syndrome.- Proteinuria (>3.5 g/day), hypoalbuminuria (→ edema), hyperlipidemia - Most commonly affects Caucasian adults → Primary (idiopathic): Autoantibodies to phospholipase A2 receptor→ Secondary: Autoantibodies generated in response to infection (hepatitis B and C), malignancy, autoimmune conditions (SLE), medications Findings:Light microscopy: Glomerular capillary loops and basement membrane appear thickenedImmunofluorescent microscopy: Granular subepithelial depositsElectron microscopy: "Spike and dome pattern" of subepithelial deposits Treatment: - Primary: Steroids- Secondary: Treat underlying disease
• Membranoproliferative glomerulonephritis Innume deposits in glomerulus → inflammation → Nephritic syndrome that co-presents with nephrotic syndrome (hematuria, hypertension, oliguria, azotemia) Type I:- Most common form- Circulating immune complexes that might form from a chronic infection (eg, hepatitis B and C) - Subendothelial deposits with granular immunofluorescence Type II: Dense deposit disease- Autoantibody nephritic factor stabilizes C3 convertase → persistent complement activation- Low levels of C3 Pathology:- Deposits in subendothelial layer (type I)- Recruit inflammatory cells which damage the endothelium- Thickening of the basement membrane- Proliferation of mesangial cells (= mesangial interposition) → splits basement membrane → "tram track" on light microscopy
• Rapidly progressive glomerulonephritis Nephritic syndrome that is characterized by epithelial cell proliferation in to a thick crescent shape.- Usually adults in 50-60s- Poor pronosis if not treated early Type I: - Anti-glomerular basement membrane antibodies- Goodpasture syndrome Type II: Immune-complex-mediated- Poststreptococcal glomerulonephritis, SLE, IgA nephropathy, Henoch-Schönlein purpura Type III: Pauci-immune- No antibodies, no immune complexes- cANCA → Wegener granulomatosis- pANCA → Microscopic polyangiitis, Churg-Strauss syndrome (granulomatous, asthma, eosinophilia) Pathology:- Glomerular basement membrane breaks → hematuria, inflammatory mediators, plasma proteins, fibrin → parietal epithelial cells proliferate → Crescent- Crescents may undergo sclerosis and be replaced by connective tissue- Types can be differentiated in immunofluorescence: Type I → linear; type II → granular; type III → negative Complications: Hematuria, decreased GFR, acute renal failure Treatment: - Glucocorticoids and cyclophosphamide- In the event of Goodpasture syndrome: plasmapheresis in addition to immunosuppression
• Extrakapillär proliferative Glomerulonephritis (Rapidly progressive glomerulonephritis) 1. Extrakapilläre Proliferate (Halbmonde): bestehen aus proliferierten Parietalepithelien und Entzündungszellen (Histiozyten, Neutrophile), Fibrin 2. Fibrinaustritt in den Kapselraum 3. Entzündliche Reaktion
• Tubulointerstitielles Infiltrat - Differentialdiagnose - Chronische Pyelonephritis - Analgetika-Nephropathie - Hydronephrosis - Transplantatabstossung
• Chronic pyelonephritis Where the kidney develops permanent scarring as a result of recurrent acute pyelonephritis. Etiology: 1. Vesicoureteral reflux (eg, failure of vesicoureteral orfice, bladder outlet obstruction)2. Chronic obstruction (eg, posterior urethral valves, benign prostatic hyperplasia, cervical carinoma, urinary calculi) Pathology:- Fibrosis and scarring of renal interstitium- Tubule atrophy- Chronic inflammatory changes such as rough scarring at the junction of the cortex and medulla- Blunted calyces from recurrent urinary reflux- Tubule dilated and filled with colloid → form casts ("thyroidization") which can show in urine Xanthogranulomatous pyelonephritis: Infected kidney stone causes crhonic obstruction- Granulomatous tissue willed with foamy (fat-laden) macrophages (DD: Kidney tumor!)
• Akute Pyelonephritis 1. Destruktion der Nierentubuli durch neutrophile Granulozyten 2. Streifenförmige Entzündungsherde vom Mark bis in die Rinde (aszendierende Pathogenese) 3. Hyperämie des angrenzenden Parenchyms
• Transplantatabstossung 1. Intimaödem mit Einengung der Gefässlichtung (akut) 2. Entzündliche Infiltration des Zwischengewebes, Untergang von Tubulusepithelien (akut) 3. Fibrotische Verlegung der Lichtung einer Nierenarterie (chronisch)
• Renal cell carcinoma Most common type of kidney cancer in adults.- Form from epithelial cells in the proximal convoluted convule of the kidney in the cortex.- Mutation in VHL gene on chromosome 3 - M > F- Peak incidence: 50-70 years Risk factors:- Lifestyle: Smoking, obesity- Hypertension- Existing kidney disease- Chemotherapeutic agents (e.g., cisplatin) during childhood - Chronic analgesic use (especially acetaminophen, and aspirin) Hereditary renal cell carcinomas: - Von Hippel-Lindau syndrome- Hereditary papillary renal cell carcinoma (HPRCC)- Tuberous sclerosis Symptoms: Flank pain, palpable mass, hematuria- Constitutional symptoms: weight loss, fatigue, night sweats, fever- Paraneoplastic syndromes (EPO → polycythemia; renin → hypertension; PTHrP → hypercalcemia; ACTH → Cushing syndrome)- If on left side with infiltration of renal vein → varicocele- Hematogenous spread → metastases to lung, bones Pathology:- Polygonal epithelial cells with angular shapes, clear cytoplasm filled with carbohydrates and lipids- Hemorrhage- Pseudocapsule- Often golden-yellow due to lipid content Grading: Fuhrmann Treatment:- RCCs are resistant to chemotherapy and radiation therapy- If localized → resection (nephrectomy)- Immunomodulatory agents (eg, aldesleukin [IL-2], interferon-α), tyrosine kinase inhibitors (eg, sorafenib), anti-VEGF antibodies (eg, bevacizumab)
• Klarzelliges Nierenzellkarzinom 1. Nekrotischer Tumor mit Blutungsherden 2. Hellzellige Tumorzellen, welche Fett und Glykogen enthalten 3. Kapillarreiches, stromaarmes Interstitium 4. Tumor bricht oft in Venen ein 5. Pseudokapsel
• Renal cell carcinoma - Subtypes Clear cell RCC (~80%)- Polygonal cells with a clear, glycogen and/or lipid-filled cytoplasm that are arranged as cords or tubules (non-papillary growth)- Unifocal, unilateral growth Papillary RCC (~10%)- Cuboidal, low columnar cells that grow in papillary formations- Bilateral, multifocal growth may occur. Chromophobic RCC (~5%)- Large polygonal cells with a prominent cell membrane, eosinophilic cytoplasm, and a perinuclear halo- Excellent prognosis Oncocytic RCC (~5%)- Originate from oncocytomas- Similar to chromophobic RCC except that there is no perinuclear halo and the cells occur as tumor nests- Excellent prognosis Collecting duct carcinoma (Bellini duct carcinoma) (∼1%)- Malignant glandular cells that are arranged as irregular within a fibrous stroma (hobnail pattern)- Aggressive tumor with a poor prognosis
• Renal mass - Differential Benign masses- Angiomyolipoma- Oncocytoma- Metanephric adenoma- Renal abscess- Granulomatous renal disease (e.g., renal tuberculosis, xanthogranulomatous pyelonephritis)- Renal cysts (e.g., polycystic kidney disease) Malignant masses- Renal cell carcinoma- Transitional cell/urothelial carcinoma of the renal pelvis (∼8% of renal tumors)- Metastasis from extrarenal tumors- Other rare primary malignancies: lymphomas, soft tissue sarcomas, carcinoid tumors
• Nephroblastom (Wilms Tumor) 1. Hohlräume ausgekleidet von einem kubischen Epithel 2. Triphasisches Muster: blastemale, epitheliale und stromale Komponente- Blastemzellen: klein, zytoplasmaarm- Epitheliale Komponente: primitive tubuläre Strukturen, manchmal auch glomeruloide Strukturen- Stromale Komponente: spindelige Zellen, Fibroblasten, glatte oder quergestreifte Muskelzellen 3. Hämorrhagien 4. Nekrosen 5. Pseudokapsel
• Wilms' tumor (nephroblastoma) Kidney tumor composed of metanephric blastema cells (give rise to stromal and epithelial cells).- Most common malignant kidney tumor in children.- Peak incidence: 2-5 years Sporadic or due to mutation on chromosome 11 (WT1/WT2 gene). Associated syndromes:WT1 gene:- WAGR syndrome: Wilms tumor and genitourinary malformation, aniridia, intellectual disability- Denys-Drash syndrome: Wilms tumor, early onset nephrotic syndrome, male pseudohermaphroditismWT2 gene:- Beckwith-Wiedemann syndrome: Wilms' tumor, macroglossia, organomegaly, hemihypertrophy Clinical presentation:- Abdominal mass that is large, non-tender, unilateral (not crossing the midline), smooth and firm- Hematuria- Hypertension Diagnostics:- Best initial test: Ultrasound- Abdominal CT/MRI- CT thorax for stagingBiopsy is usually reserved for assessing nodules that are suspected metastases, as tumor capsule rupture and spillage results in more advanced staging and intensive treatment! Pathology:- Triphasic blastoma: Blastemal, stromal and epithelial cels- Abortive structures: glomerular and tubules- May include cysts, hemorrhage, or necrosis- Pseudocapsule Treatment: - Nephrectomy + Chemotherapy (dactinomycin and vincristine)- Good prognosis
• Urothelial cancer Urothelial cell carinoma that can affect the renal pelvis, ureter, bladder and urethra. - Most commony in the urothelium of the bladder.- M > F- Peak incidence: 65 years- Cancer sites:→ Bladder (90%)→ Renal pelvis (8%)→ Ureter (esp. the lower ⅓) and urethra (2%) Urothelium is composed of 3 to 7 cell layers. Forms a tight barrier layer by umbrella cells and tight junctions. Have the ability to stretch with an extending bladder. Histological types:- Transitional cell carcinoma: most common (∼95%) type of cancer of the bladder, ureter, renal pelvis, and proximal urethra in males- Squamous cell carcinoma: most common (∼60%) type of cancer of the distal urethra in males and the entire urethra in females Risk factors: Age, carcinogens (phenacetin, smoking, aniline, cyclophosphomide, alcohol abuse), chronic inflammation of the urinary tract (e.g., chronic/recurrent UTI, schistosomiasis)  Symptom: - Painless hematuria throughout micturition- Irritative voiding symptoms (dysuria, urinary frequency, urgency) Diagnosis: - CT urography- Cystoscopy and biopsy→ ulcerations; tumor cells are large and have large, bright nuclei; infiltration of the detrusor muscle; umbrella cells no longer defined Treatment: - Difficult because they are often multifocal and can recur after treatment- Some can be resected with transurethral resection (TUR) + chemotherapy from catheter (= intravesicle chemotherapy)- Aggressive cancer → cysto-prostatectomy + chemotherapy
• Bladder cancer Urothelial cell carcinoma (= Transitional cell carcinomas) Non-urothelial cell carinoma:- Squamous cell carcinoma (arise from squamous cell metaplasia)→ Typically form in multiple locations→ Keratinization→ Chronic irritation: UTIs, kidney stones, schistosoma haematobium- Adenocarcinoma→ More rare but frequently metastasize→ Solitary, derive from glandular tissue → produce mucins→ Main form of tumor in patients with bladder extrophy→ Common with schistosoma hematobium infections Diagnosis: Cystoscope + biopsy Treatment: Transurethral resection or radical cystectomy
• Renal cortical necrosis Irreversible type of prerenal acute renal injury caused by a sudden drop of blood perfusion to the renal cortex.- Cortical radial arteries are end arteries and therefore more susceptible to ischemia. Causes: Reduced blood flow, thrombi, vasospasm- Associated with pregnancy complications. Ischemia → Inflammation → Fluid leak into interstitium → vasoconstriction of afferent arteriole → decreases GFR.Proximal tubule and thick ascending loop of Henle require more energy and thus the first to start dying and detaching. Dead cells can clog nephron → increased nephron pressure → decreases GFR. → Acute tubular necrosis: If the obstruction goes away; the damage to the kidney is generally reversible.→ Irreversible necrotic injury: If ischemia persists. Symptoms:- Sharp decrease in urine output- Kidney swells up → flank pain Diagnosis: - Blood studies: Excess BUN, creatinine, hyperkalemia, metabolic acidosis- Urine: Hematuria, proteinuria, tubular cell casts- CT scan: Nonenhancing renal cortex; sometimes with thin rim of contrast enhancement- Biopsy: Cortex shows patchy necrosis and atrophy while medulla looks normal Treatment: Increase blood transfusion
• Oncocytoma Benign tumor arising from the intercalated tubular cells.An oncocytoma is not confined to the kidneys and may develop in the thyroid gland, pancreas, or the pituitary gland. Pathology- Macroscopy: brown tumor with central radial scar- Microscopy: excessive amount of mitochondria → acidophilic, granular cytoplasm- No perinuclear clearing (vs. chromophobic RCC). Therapy: Surveillance- If tumor increases in size → suspicious for malignant transformation in RCC → nephrectomy- Often resected in order to exclude RCC Prognosis: Oncocytomas are not invasive, but they may transform into a malignant oncocytic RCC.
• Angiomyolipoma Benign renal tumors that arise from perivascular epithelioid cells and consist of blood vessels, smooth muscle, and mature fat cells. - Mean age of onset: 43 years- Sex: F > M (4:1)- More often on right kidney Etiology- Sporadic- May be associated with the following syndromes: Tuberous sclerosis, sporadic lymphangioleiomyomatosis Clinical features- Mostly asymptomatic- Large angiomyolipomas may present with hematuria, retroperitoneal hemorrhage, and impaired renal function. Diagnostics:- Abdominal ultrasound: round, well-circumscribed, highly echogenic (similar echogenicity to renal pelvis) renal tumor often located near the renal capsule- Abdominal CT: tumor with macroscopic fat deposits, no calcification- Percutaneous biopsy may be required if imaging is inconclusive Treatment: Surgical resection of the tumor is indicated for angiomyolipomas that measure more than 4 mm in diameter.
• Acute tubular necrosis Causes ∼85% of intrinsic AKIs. - The straight segment of the proximal tubule and the thick ascending limb are especially susceptible to ischemic damage in acute tubular necrosis. Etiology:- Ischemic: Prolonged hypovolemia/shock, thromboembolism, thrombotic microangiopathy, cholesterol embolism (atheroemboli)Toxic: - Contrast-induced nephropathy - Medication: aminoglycosides, cisplatin, ethylene glycol- Pigment nephropathy: Myoglobinuria due to rhabdomyolysis (crush syndrome) Clinical presentation: 3 stages1. Inciting event2. Maintenance phase — oliguric; lasts 1-3 weeks; risk of hyperkalemia, metabolic acidosis, uremia3. Recovery phase — polyuric; BUN and serum creatinine fall; risk of hypokalemia and renal wasting of other electrolytes and minerals Urinary sediment: - Brownish ("muddy") granular epithelial cell casts- Renal tubular epithelial cells (due to denudation of tubular basement membrane) Pathology:- Degenerative Tubuli (Koagulationsnekrose)
• Acute interstitial nephritis (tubulointerstitial nephritis) Kidney injury most commonly caused by a hypersensitivity reaction to drugs (allergic interstitial nephritis). Some drugs may also lead to crystal-induced acute kidney injury. Allergic interstitial nephritis:- Etiology: Antibiotics (penicillins and cephalosporins), NSAIDs, diuretics, PPIs, rifampin- Diagnostics: Pyuria, eosinophiluria- Histology: diffuse interstitial inflammatory infiltrate (T lymphocytes, monocytes) Crystal-induced acute kidney injury:- Etiology: Acyclovir, indinavir, ciprofloxacin, methotrexate- Diagnostics: Crystals on brightfield microscopy Symptoms- Painless hematuria- Flank pain- Sterile pyuria- Rash- Fever- Arthralgias Therapy: Discontinue drugs and administer IV fluids
• Pyelonephritis Infection of the renal pelvis and parenchyma that is usually associated with an ascending bacterial infection of the bladder. Pathogens:- Escherichia coli (∼75-90% of cases) - Pseudomonas aeruginosa- Klebsiella pneumoniae- Proteus mirabilis Risk factors:- Women because they have shorter urethras- Pregnancy- Urinary tract obstruction: Foreign bodies, anatomical abnormalities (benign prostatic hyperplasia, vesicoureteral reflux, nephrolithiasis, ureteral strictures)- Cystitis- Recent administration of antibiotics (possible antibiotic resistance)- Immunosuppression (eg, HIV, diabetes) Clinical presentation:- High fever, chills, flank pain, dysuria as well as other symptoms of cystitis (eg, frequency, urgency) Diagnostics: - Urinalysis: Pyuria (→ positive esterase on dipstick test), leukocyturia, WBC casts, bacteriuria (→ positive nitrites on dipstick test), microhematuria- Urine culture and blood culture Pathology:Destructive interstitial nephritis: - Purulent inflammation of the interstitium with destruction of the parenchyma, the renal tubules, and in some cases the renal pelvis- Renal tubules infiltrated with neutrophilsChronic pyelonephritis: - Chronic inflammatory changes such as rough scarring of at the junction of the cortex and medulla- Blunted calyces from recurrent urinary reflux- Eosinic (not eosinophilic!) casts in the tubules (so called “thyroidization of the kidney”)Xanthogranulomatous pyelonephritis:- Lipid laden foamy macrophages and multinucleated giant cells seen on histology- Large, irregular, yellow appear masses throughout the kidney on gross examination Treatment:- Uncomplicated: oral fluoroquinolones (eg, ciprofloxacin for 7 days)- Complicated: IV fluoroquinolones (eg, ciprofloxacin) for 10-14 days
• Microscopic polyangiitis Necrotizing vasculitis of small vessels, typically with renal and pulmonary involvement; however, multiple organ systems may be affected. Clinical features: - Similar to that of granulomatosis with polyangiitis, but without involvement of the nasopharynx- Renal: Glomerulonephritis with hypertension has a poor prognosis- Lungs: pulmonary vasculitis with hemoptysis- Skin: palpable purpura, nodules, necrosis Diagnostics:- Biopsy of involved organ: fibrinoid necrosis with infiltration of neutrophils; no granulomas- Laboratory findings: MPO-ANCA/pANCA (∼70% of cases) Treatment: Corticosteroids and cyclophosphamide

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