Chapter 340 Tubuloinyerstitial Diseases of The Kidney Flashcards
The acute nature of this group of disorders may be caused by aggressive inflammatory infiltrates that lead to tissue edema, tubular cell injury, and compromised tubular flow, or by frank obstruction of the tubules with casts, cellular debris, or crystals.
Acute TIN
(glycosuria, phosphaturia, aminoaciduria, hypokalemia
Fanconi’s syndrome
predominant pathology includes
interstitial fibrosis with patchy mononuclear cell infiltration and widespread tubular atrophy, luminal dilation, and thickening of tubular basement membranes
chronic TUBULOINTERSTITIAL nephritis CTIN
accounts for no more than ~15% of cases of unexplained acute renal failure
Allergic interstitial nephritis
classic presentation , namely, fever, rash, peripheral eosino- philia, and oliguric renal failure occurring after 7–10 days of treatment with methicillin or another β-lactam antibiotic, is the exception rather than the rule.
Allergic interstitial nephritis
In AIN, Atypical reaction can occur most notably by ____ which fever, rash, and eosinophilia are rare, but acute renal failure with heavy proteinuria is common.
NSAID INDUCED AIN
In AIN, Urinalysis reveals
pyuria with white blood cell casts and hematuria.
systemic autoimmune disorder that primarily targets the exocrine glands, especially the lacrimal and salivary glands, and thus results in symptoms, such as dry eyes and mouth, that consti- tute the “sicca syndrome”
SJÖGREN’S SYNDROME
is the most common renal mani- festation of Sjögren’s syndrome
Tubulointerstitial nephritis with a predominant lymphocytic infiltrate
associated with distal RTA, nephrogenic diabetes insipidus, and moderate renal failure
SJÖGREN’S SYNDROME
Sjögren’s syndrome Diagnosis is strongly supported by
positive serologic testing for anti-Ro (SS-A) and anti-La (SS-B) antibodies.
A large proportion of patients with Sjögren’s syndrome also have
polyclonal hypergammaglobulinemia
Treatment of SJS initially with glucocorticoids, although patients may require maintenance therapy with ____ to prevent relapse
azathioprine or mycophenolate mofetil
is a systemic autoimmune disease of unknown etiology. It accounts for fewer than 5% of all cases of AIN, affects females three times more often than males, and has a median age of onset of 15 years.
TINU
hallmark feature of TUBULOINTERSTITIAL NEPHRITIS WITH UVEITIS (TINU) in addition to a lymphocyte-predominant interstitial nephritis is
a painful anterior uveitis, often bilateral and accompanied by blurred vision and photophobia.
Diagnoses is often confounded by the fact that the ocular symptoms precede or accompany the renal disease in only one-third of cases.
TUBULOINTERSTITIAL NEPHRITIS WITH UVEITIS (TINU)
Additional extrarenal features include fever, anorexia, weight loss, abdominal pain, and arthralgia. The presence of such symptoms as well as elevated creatinine, sterile pyuria, mild proteinuria, features of Fanconi’s syndrome, and elevated erythrocyte sedimentation rate should raise suspicion for this disorder.
TINU
TINU:
The renal and ocular manifestations generally respond well to oral glucocorticoids, although maintenance therapy with agents such as ____may be necessary to prevent relapses
methotrexate, azathioprine, or mycophenolate
Some patients may present with features of AIN but follow a pro- tracted and relapsing course.
GRANuLOMATOuS INTERSTITIAL NEPHRITIS
Renal biopsy in such patients reveals a more chronic inflammatory infiltrate with granulomas and multinucleated giant cells.
GRANuLOMATOuS INTERSTITIAL NEPHRITIS
In GRANuLOMATOuS INTERSTITIAL NEPHRITIS
Most often, no associated disease or cause is found; however, some of these cases may have or subsequently develop the pulmonary, cutaneous, or other systemic manifestations of
sarcoidosis such as hypercalcemia.
is a rare cause of granulo- matous interstitial nephritis.
Tuberculosis
Autoimmune pancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, and a chronic scleros- ing sialadenitis (mimicking Sjögren’s syndrome) may variably be pres- ent as well.
IgG4 related systemic disease
Fibrotic lesions that form pseudotumors in the affected organs soon replace the initial inflammatory infiltrates and often lead to biopsy or excision for fear of true malignancy.
IgG4 related systemic disease
Presently, infection-associated AIN is most often seen in immunocompromised patients, particularly
renal transplant recipients with reactivation of polyomavirus BK
Urinalysis reveals “sheaf of wheat” sulfonamide crystals,
tubular obstruction that may occur in patients treated with sulfadiazine for toxoplasmosis,
Ua reveals individual or parallel clusters of needle- shaped indinavir crystals
Tubular obstruction from indinavir and atazanavir for HIV
Urinalysis showing red-green birefringement needle-shaped crystals of acyclovir.
Tubular obstruction from intravenous acyclovir for severe herpesvirus infections.
A dense precipitate of birefringent uric acid crystals is found in the urine, usually in association with microscopic or gross hematuria
acute urate nephropathy
is an uncommon but serious complication of oral Phospho-soda used as a laxative or for bowel preparation for colonoscopy.
Acute phosphate nephropathy
commonly known as myeloma kidney—should be considered in patients who fail to recover when the precipitating factor is corrected or in any elderly patient with other- wise unexplained acute renal failure.
light chain cast nephropathy (LCCN)
In this disorder, filtered monoclonal immunoglobulin light chains (Bence-Jones proteins) form intratubular aggregates with secreted Tamm-Horsfall protein in the distal tubule
light chain cast nephropathy (LCCN)
Clinical clues to the diagnosis include anemia, bone pain, hypercalcemia, and an abnormally narrow anion gap due to hypoalbuminemia and hypergammaglobulinemia.
light chain cast nephropathy (LCCN)
detection of increased amounts of protein in a spot urine specimen and a negative dipstick result are highly suggestive that the urine contains
Bence-Jones protein
is the consequence of vesicoureteral reflux (VUR) or other urologic anomalies in early childhood
Reflux nephropathy
stems from abnor- mal retrograde urine flow from the bladder into one or both ureters and kidneys because of mislocated and incompetent ureterovesical valves
vesicoureteral reflux (VUR)
In VUR, result is patchy interstitial scarring and tubular atrophy. Loss of functioning nephrons leads to hypertrophy of the remnant glomeruli and eventual secondary
FSGS
Renal ultrasound in adults characteristically shows asymmetric small kidneys with irregular outlines, thinned cortices, and regions of compensatory hypertrophy
vesicoureteral reflux (VUR)
In VUR, Aggressive control of blood pressure with _____ is effective in reducing proteinuria and may significantly forestall further deterioration of renal function.
an angiotensin-con- verting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) and other agents
In 1. _____ there is evidence of tubular injury and may be evident in childhood and early adolescence in the form of 2.____ due to decreased concentrating ability or 3. ______ years before there is significant nephron loss and proteinuria from secondary FSGS.
- Sickle cell nephropathy
- Polyuria
- type IV renal tubular acidosis (RTA)
MajOr Causes OF papILLary neCrOsIs
analgesic nephropathy
Sickle cell nephropathy
Diabetes with urinary tract infection
Prolonged NSAID use (rare)
results from the long-term use of compound analgesic preparations containing phenacetin (banned in the United States since 1983), aspirin, and caffeine.
analgesic nephropathy
most prudent approach in lithium associated nephropathy is to monitor lithium levels frequently and adjust dosing to avoid toxic levels
<1 meq/L)
in lithium associated nephropathy, patients who develop significant proteinuria, ____ treatment should be initiated
ACEI or ARB
calcineurin inhibitor (CNI) immunosuppressive agents ____ can cause both acute and chronic renal injury.
cyclosporine and tacrolimus
is typically seen in solid organ (including heart-lung and liver) transplant recipients and manifests with a slow but irreversible reduction of glomeru- lar filtration rate, with mild proteinuria and arterial hypertension.
Chronic CNI-induced renal injury
is a relatively common complication of calcineurin-inhibitor nephropathy and is caused, in part, by tubular resistance to aldosterone.
Hyperkalemia
triad of “saturnine gout,” hypertension, and renal insufficiency attributable to proximal tubule dysfunction
HEAVY METAL (LEAD) NEPHROPATHY
a form of medullary cystic kidney disease caused by mutations in uromodulin (UMOD)
juvenile hyperuricemic nephropathy
Chronic hypercalcemia, as occurs in ___(5)____, can cause tubulointerstitial disease and progressive renal failure
primary hyperparathyroidism, sarcoidosis, multiple myeloma, vitamin D intoxication, or metastatic bone disease
Clinically, the most striking defect is an inability to maximally concentrate the urine, due to reduced collecting duct responsiveness to arginine vasopressin and defective transport of sodium and chloride in the loop of Henle.
hypercalcemic nephropathy
Analgesic nephropathy results from the long-term use of compound analgesic preparations containing:
- phenacetin (banned in the United States since 1983)
- aspirin
- caffeine
Two seemingly unrelated forms of CIN, Chinese herbal nephropathy and Balkan endemic nephropathy, have recently been linked by the underlying etiologic agent aristolochic acid and are now collectively termed
aristolochic acid nephropathy (AAN)
Definitive diagnosis of AAN requires two of the following three features:
- characteristic histology on kidney biopsy;
- confirmation of aristolochic acid ingestion;
and - detection of aristolactam-DNA adducts in kidney or urinary tract tissue.
It has been linked to mutations in FAN1, a nuclease involved in DNA repair, which may render carriers of the mutation susceptible to environmental DNA-damaging agents.
Karyomegalic interstitial nephritis
