Nephritic Syndromes

Question 1

List 3 Immune Complex Mediated Nephritic Syndromes

Answer 1

1. ) Postinfectious Glomeruloephritis
2. ) IgA Nephropathy (Berger Disease)
3. ) Henloch-Shonlein Purpura

Question 2

List 3 Pauci-Immune Mediated Nephritic Syndromes

Answer 2

1. ) Granulomatosis with Polyangitis, GPA, (Wegner’s)
2. ) Microscopic Polyangitis
3. ) Eoisinophilic Granulomatosis with Polyangitis

Question 3

List 2 Anti-GBM Mediated Nephritic Syndromes

Answer 3

1. ) Goodpasture Syndrome

2. ) Alport Syndrome

Question 4

Describe the Hx, Labs, and Treatment/Prognosis of:

Postinfectious Glomerulonephritis

Answer 4

Description/Hx: 2-6 weeks following GBS infection (or any infection). Oliguria, tea or cola-colored urine.

Labs: Low Serum C3, ASO and anti-dsDNA Abs. “Lumpy/Bumpy” IF.

Tx/Prognosis: Supportive care, diuretics to prevent overload, most make full recovery

Question 5

Describe the Hx, Labs, and Treatment/Prognosis of:

IgA Nephropathy

Answer 5

Description/Hx: Most common type. Typically seen in young men. Follows URI or GI infection. Features episodic gross hematuria or microscopic hematuria.

Labs: Normal C3, contrast to PIGN

Tx/Prognosis: Glucocorticoids, ACEI. 20% of cases progress to ESRD

Question 6

Describe the Hx, Labs, and Treatment/Prognosis of:

Henloch-Shonlein Purpura

Answer 6

Description/Hx: Small-vessel vasculitis seen in childhood. Triad of palpable purpura, arthralgias, and abdominal pain.

Labs: Normal C3, compare to IgA neph and contrast with PIGN

Tx/Prognosis: Supportive therapy + glucocorticoids

Question 7

Describe the Hx, Labs, and Treatment/Prognosis of:

Granulomatosis with Polyangitis (GPA)

Answer 7

Description/Hx: Granulomatous infl. of respiratory tract with nasopharyngeal involvment and kidney nec. vasculitis. Respiratory and sinus symptoms, pulmonary lesions bleed and lead to hemoptysis.

Labs: c-ANCA, segmental necrotizing glomerulonephritis

Tx/Prognosis: High-dose corticosteriods, cytotoxic agents, rituximab. Frequent relapses

Question 8

Describe the Hx, Labs, and Treatment/Prognosis of:

Microscopic Polyangitis

Answer 8

Description/Hx: Small-vessel vasculitis similar to GPA but lacking granulomas and nasopharyngeal involvement.

Labs: p-ANCA

Tx/Prognosis: Glucocorticoids, cyclophosphamide, rituximab

Question 9

Describe the Hx, Labs, and Treatment/Prognosis of:

Eosinophilic Granulomatosis with Polyangitis

Answer 9

Description/Hx: Small-vessel vasculitis. Presents with asthma, skin nodules/purpura, peripheral neuropathy

Labs: p-ANCA, IgE (think ASTHMA)

Tx/Prognosis: Glucocorticoids

Question 10

Describe the Hx, Labs, and Treatment/Prognosis of:

Goodpasture Nephritis

Answer 10

Description/Hx: Anti-GBM. Rapidly-progression glomerulonephritis with pulmonary hemorrhage. Peak in men in mid-20’s. Hemoptysis, dyspnea, respiratory failure possible, no URT involvment.

Labs: Linear anti-GBM dep. on IF, Iron-def. anemia, hemosiderin-laden macs in sputum, pulmonary infiltrates on CXR.

Tx/Prognosis: Plasma exchange therapy

Question 11

Describe the Hx, Labs, and Treatment/Prognosis of:

Allport Nephritis

Answer 11

Description/Hx: Anti-GBM, hereditary glomerulonephritis. Present in boys 5-20 yo. Asymptomatic hematuria associated with sensorineural deafness and eye disorders.

Labs: GBM-splitting on IF

Tx/Prognosis: Progresses to renal failure, may recur after transplant