Nephro- Glomerular diseases

What are typical findings of glomerular diseases presenting as nephritic syndrome?

Hematuria, proteinuria, HTN, reduced GFR

What is one of the most helpful techniques available to define cause of glomerular disease (but not done initially)?

Renal biopsy

What are other names for nephritic syndrome?

Acute glomerulonephritis, nephritis syndrome

What is a group of disorders that cause inflammation of internal kidney structures, specifically the glomeruli, & is often caused by immune response triggered by infx or other dz?

Nephritic syndrome

What are the essentials of diagnosis for nephritic syndrome?

Edema, HTN, hematuria (± dysmorphic RBCs, RBC casts)

The following ssx are seen with what condition?

• Dark, cola colored urine & decreased urine volume

• Edema first in regions of low tissue pressure (periorbital, scrotum)

• HTN d/t volume overload (check JVD & adventitious lung sounds)

Nephritic syndrome

What would you see in a UA in a patient with nephritic syndrome?

Dark cola colored urine, ± dysmorphic RBCs, RBC casts, proteinuria

What is the treatment for nephritic syndrome?

Reduce glom inflammation, admit most pts

Aggressive reduction HTN/fluid → Na & H2O restriction, diuretics, dialysis

Inflammatory glomerular injury → corticosteroids, cytotoxic agents

Who has a better prognosis in nephritic syndrome, children or adults?

Children

What causes postinfectious glomerulonephritis (GN)?

GAS

How does a patient present with post infectious GN?

Oliguric, edematous, & variable hypertensive

What labs may be seen in post infectious GN?

Elevated ASO titers, positive throat culture, cola colored urine, urinary RBCs, red cell casts, and proteinuria < 3.5 g/day

What is the treatment for post infectious GN?

Supportive, bedrest, abx, antihypertensive, salt restriction, diuretics PRN

What is the prognosis for children with post infectious GN?

Most fully recover w/ normal renal studies w/in 2 mos

What is the prognosis for adults with post infectious GN?

Prone to crescent formation and chronic renal insufficiency

What is another name for IGA nephropathy?

Berger’s disease

What condition is a primary renal dz of IgA deposition in the glomerular mesangium, frequently associated with a URI or flu like illness, & is the MC form of acute GN?

IGA nephropathy

What is the most common presenting complaint with IGA nephropathy?

Episodes of painless hematuria

What is the diagnosis for IgA nephropathy?

Elevated serum IgA &r enal bx (gold standard) → focal GN w/ diffuse mesangial IgA deposits and proliferation of mesangial cells in glomeruli

What is the treatment for IGA nephropathy?

Bedrest

If proteinuria > 1g/day → ACEi or ARBs (target BP < 125/75), add steroids if persists

What condition is a leukocytoclastic vasculitis of unknown cause MC in young males under 20 y/o?

IgA vasculitis

What is another name for IgA vasculitis?

Henoch-Schonlein purpura

How does Iga vasculitis present?

Palpable purpura (MC on LE), arthralgias, abd sx - N, colic, melena, & renal insufficiency w/ nephritic presentation

What is the treatment for IgA vasculitis?

Bedrest, +/- steroids, most fully recover over several weeks

How does rapidly progressive GN (RPGN) present?

Acute onset → zotemia, oliguria, edema, HTN, proteinuria, & hematuria w/ active urine sediment that contains RBC or pigmented casts & cellular debris

≥ 50% loss of renal function w/in wks-mos

If renal failure in RPGN is severe, what other manifestations may appear?

Uremia, N/V, hiccups, dyspnea, lethargy, pericarditis, encephalopathy, CHF & pulm edema from severe volume overload

What are possible causes of RPGN?

SLE, cryoglobulinemia, HCV, subacute infective endocarditis, shunt infx, good pasture’s syndrome, granulomatosis w/ polyangiitis

When is RPGN likely?

> 50% of glomeruli contain crescents

What are crescents?

Proliferation of cells within bowman’s capsule in response to glomerular rupture (marker of severe injury)

What condition is defined by clinical constellation of GN and pulmonary hemorrhage, mediated by anti-GBM antibodies?

Goodpasture’s syndrome

How do patients with goodpasture’s syndrome present?

Recent hemoptysis, tachypnea, malaise, anorexia, HA, HTN, edema, ± preceding URI

What is the diagnosis for goodpasture’s syndrome?

Urine → gross or microscopic hematuria

Bun/Cr → severely suppressed renal function

CXR → pulm infiltrates d/t hemorrhage

Bx → glom crescents, adhesions, & interstitial inflammatory infiltration

Confirm dx w/ circulating anti-GBM

What is the treatment for goodpasture’s syndrome?

Admit, large dose corticosteroids + immunosuppressive therapy, plasmapheresis, transplant (delay until antibodies have disappeared)

What condition is characterized by heavy proteinuria (> 3.5 g/day), hypoalbuminemia, edema, & hypercholesterolemia?

Nephrotic syndrome

What can occur in severe cases of nephrotic syndrome?

Venous thrombosis

What are the essentials of diagnosis for nephrotic syndrome?

Urine protein > 3.5 g/day, hypoalbuminemia < 3 g/dL, & peripheral edema

What are ssx of nephrotic syndrome?

Peripheral edema (hallmark) occurring when albumin < 3 g/dL, & dyspnea d/t pulm edema, pleural effusions, & diaphragmatic compromise w/ ascites

The following labs are seen with what condition?

• Proteinuria as result of alteration of negative charge in GBM

• dec serum albumin < 3

• hyperlipidemia → lipiduria (oval fat bodies in urine, grape clusters / maltese cross)

• hypertriglyceridemia

• microscopic hematuria possible but not likely

Nephrotic syndrome

How do oval fat bodies in urine (lipiduria) appear under light microscopy?

Grape clusters or maltese cross

When would a renal bx be used in nephrotic syndrome?

Determine prognosis & treatment, r/o primary cause requiring drug therapy in adults, occasional unexpected dx

*don’t do w/ extremely high Cr (irrev renal dz)

What is the treatment for nephrotic syndrome?

Proteinuria → ACEi, ARB, dietary consult, monitor loss & intake

Edema → salt restriction, thiazide & loop diuretics

HLD → diet, exercise, statins

What is the treatment to prevent thromboemboli in nephrotic patients with serum albumin < 2 g/dL who can become hypercoagilable?

IV albumin, long term anticoagulation therapy

What are the 4 most common causes of nephrotic syndrome?

Minimal change dz, membranous nephropathy, focal glomerular sclerosis, membranoproliferative GN

What is another name for minimal change disease?

Lipoid nephrosis

What is the MCC of primary nephrotic syndrome in children the presents with the manifestations of nephrotic syndrome?

Minimal change disease

What are etiologies of minimal change disease?

Idiopathic, occur after viral URI, in association with tumors (hodgkin’s), drugs, & hypersensitivity rxns (NSAIDs & bee stings)

When a biopsy be performed in minimal change disease (MCD)?

Reserved for pts who are first treated w steroids & are steroid resistant

What is the treatment for minimal change disease (MCD)?

Prednisone

What is considered diagnostic for MCD?

Disappearance of proteinuria with prednisone

What is the MCC of primary nephrotic syndrome in adults, MC 5th and 6th decades (almost always after 30) & is associated with occult carcinoma & thromboembolism?

Membranous nephropathy

What are ssx of membranous nephropathy?

Signs of nephrotic syndrome, higher incidence of renal vein thrombosis

Higher incidence of occult neoplasms of lung, stomach, colon in pts > 50 y/o

How is membranous nephropathy dx confirmed?

Renal biopsy

What is the treatment of membranous nephropathy?

ACEi for proteinuria, transplant

What is the prognosis for membranous nephropathy?

Progressive renal failure over 5-15 yrs

What condition can present as idiopathic or secondary to heroin abuse, morbid obesity, HIV infx, & NSAID use?

Focal segmental glomerular sclerosis

How does focal segmental glomerular sclerosis present?

Microscopic hematuria, HTN, progress to ESRD in 6-8 yrs

What is the treatment for focal segmental glomerular sclerosis?

Long term oral corticosteroids

What condition, in it’s primary form, is an idiopathic syndrome that can present with nephritic or nephrotic features, MC in pts < 30 y/o & lupus nephritis patients?

Membranoproliferative NG (MPGN)

Which subtype of MPGN is more common, type I or II?

Type II

What is the treatment for MPGN?

Corticosteroids, antiplt drugs

*both types recur after renal transplant

What is the MCC of ESRD in the US?

Diabetic nephropathy (MC T1DM)

What is the MC lesion in diabetic nephropathy?

Diffuse glomerulosclerosis

What should be included in initial screening of diabetics?

UA for microalbuminemia → 24 hr collection > 30 mg/d (standard) or early morning spot albumin or albumin - cr ratio

*dipstick not sensitive enough to quantify

What is the treatment of diabetic nephropathy?

Strict glycemic control, ACEi or ARBs to control HTN

*Poor prog once dialysis begins, more susceptible to ARF from contrast material

What is a systemic autoimmune dz in which renal involvement is common, presenting with either nephritic or nephrotic syndrome or asx renal disease?

SLE

What should all SLE patients have done?

Routine UA to monitor for hematuria or proteinuria

What is the mainstay of tx for SLE?

Corticosteroids

What condition is a form of small to medium size vasculitis, MC in males, and may involve renal vessels?

Polyarteritis nodosa (PAN)

How does PAN present?

Mononeuritis, testicular pain, abd pain (usually spares lungs)

What is the dx and tx for PAN?

Bx to confirm, tx w/ corticosteroids

What is an uncommon disorder consisting of hemolytic anemia, thrombocytopenia, and ARF?

Hemolytic uremia syndromeH

How do labs appear in hemolytic uremia syndrome?

Hematuria, proteinuria, and RBC fragmentation on peripheral blood smear (obtain kidney bx for dx)

What is the treatment for hemolytic uremia syndrome?

Supportive for children, plasmapheresis for adults

What condition?

• primary → not assoc with systemic dz or assoc with MM

• secondary → d/t chronic dz such as RA, IBD, chronic infx

• degree of proteinuria not assoc w extent of renal lesions

• tx: transplant if secondary

Amyloidosis

How does HIV associated nephropathy present?

Nephrotic syndrome in pts w/ HIV (MC focal glomerulosclerosis)

What condition is a malignancy of plasma cells, primarily in older adults, with a median survival ~ 3 years?

Multiple myeloma (MM)

How does MM with renal involvement present?

Proteinuria primarily due to light chains not detected on dipstick, which mainly detects albumin

What is the treatment for MM?

Melphalan

What is renal dysfunction associated with sickle cell dz most commonly due to?

Sickling of RBCs in renal medulla

How does renal dysfunction associated with sickle cell present?

Congestion & stasis leads to hemorrhage, interstitial inflammation & papillary infarcts; hematuria, progression to ESRD

What is the classic renal manifestation of tuberculosis?

Microscopic pyuria w/ sterile urine culture (sterile pyuria)

*resolve by treating TB

Patients with proximal tubular dysfunction have decreased excretion of uric acid and are therefore more prone to what?

Gouty attacks

What is the treatment for gouty attacks?

Rx to reduce serum uric acid levels, aggressive hydration, avoid food/drugs causing hyperuricemia,