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1

What is the most accurate test for Wegener granulomatosis?

The most accurate test for WG is a biopsy of the kidney or lung looking for granulomas. Biopsy of the nasal septum is less sensitive and has more false negative results.

2

What is the treatment of Wegener's granulomatosis?

Cyclophosphamide and steroids.

3

What is Churg–Strauss syndrome?

Characterized by lung involvement, neuropathy, skin lesions, GI, cardiac, and renal involvement.

4

What is the presentation of Churg–Strauss syndrome?

Fever, weight loss, malaise. Asthma, eosinophilia, atopic diseases. Elevated eosinophil, positive P–ANCA or antimyeloperoxidase. Most accurate test is lung biopsy: granulomas, eosinophils.

5

What is the treatment of Churg–Strauss syndrome?

Glucocorticoids and cyclophosphamide.

6

What is Goodpasture syndrome?

Idiopathic disorder of renal and lung disease characterized by a unique anti–basement membrane antibody. GP does not affect multiple organs or sites in the body other than the lung and the kidney.

7

What are the clinical signs of Goodpasture syndrome?

Hematuria, proteinuria, hemoptysis, cough, dyspnea. Best test is anti–basement membrane Ab to type IV collagen. Lung or kidney biopsy shows hemosiderin macrophages and linear deposits.

8

What is the treatment of Goodpasture syndrome?

Plasmapheresis and steroids. Cyclophosphamide.

9

What is polyarteritis nodosa?

Systemic vasculitis of small and medium arteries of every organ exception lung. Renal involvement manifests as HTN, renal insufficiency, and hemorrhage due to microaneurysms. Biopsy.

10

What is the treatment of polyarteritis nodosa?

Cyclophosphamide and steroids.

11

What are the symptoms of polyarteritis nodosa?

Fever, weight loss, malaise. Involvement of skin, eyes, muscles, GI, heart, kidneys, and neurologic system. Abdominal pain and joint pain. Anemia and an elevated sedimentation rate.

12

What is the treatment of polyarteritis nodosa?

Steroids and cyclophosphamide.

13

What is Henoch–Schönlein purpura?

Systemic deposition of IgA, resulting in renal insufficiency, skin lesions, GI symptoms.

14

What is the tetrad of Henoch–Schönlein purpura?

Tetrad of palpable purpura, arthralgias, abdominal pain, and renal disease. IgA deposited in walls of blood vessels. Biopsy is usually not performed. HSP most often resolves spontaneously.

15

What is the treatment of Henoch–Schönlein purpura?

Treatment is supportive. Steroids can be used.

16

What is IgA nephropathy (Berger disease)?

IgA nephropathy with mild hematuria that resolves spontaneously in 30%. About 40–50% of patients progress to end stage renal disease. Deposition of IgA only in kidney.

17

What are the signs of Berger disease?

HTN. Usually an Asian patient under 35 years of age who has had a recent viral illness or pharyngitis, then develops hematuria 1–2 days later.

18

How is Berger disease diagnosed?

The diagnosis is based on finding IgA deposited in the kidney on biopsy.

19

What is the treatment of Berger disease?

Proteinuria is treated with ACE inhibitors or angiotensin receptor blockers (ARB).

20

What are the causes of postinfectious glomerulonephritis?

Group A beta–hemolytic streptococci (S pyogenes) and other infections can cause postinfectious glomerulonephritis. Any infectious agent, including hepatitis B and C, CMV, staph endocarditis.

21

What type of infection can cause post–streptococcal glomerulonephritis?

Poststreptococcal glomerulonephritis can occur with throat or skin infection with S pyogenes. Rheumatic fever only occurs after pharyngitis in 10–15% of patients with GABS pharyngitis.

22

What are the signs of postinfectious glomerulonephritis?

Smoky, cola urine from hematuria, red cell casts, proteinuria. Periorbital edema and HTN. The best initial test is antistreptolysin (ASO) and the antihyaluronic acid. Complement low. Renal biopsy rarely.

23

What is the treatment of postinfectious glomerulonephritis?

Treatment is management of fluid overload and HTN with diuretics. Resolves spontaneously. Antibiotics should be given to eradicate the organism from the pharynx.

24

What are thrombotic thrombocytopenic purpura and hemolytic uremic syndrome?

TTP and HUS are two different varieties of the same disease of hemolytic anemia, uremia, and thrombocytopenia.

25

What are the causes of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome?

TTP is associated with HIV, but it is most often idiopathic. HUS is more common in children and with E. coli 0157:H7 food poisoning.

26

What is the triad of hemolytic uremic syndrome?

HUS is a triad of hemolytic anemia, uremia, and thrombocytopenia.

27

What are the signs of thrombotic thrombocytopenic purpura?

TTP has anemia, uremia, and thrombocytopenia, and is also associated with fever and headache, confusion, seizures, focal deficit.

28

What are the laboratory findings in hemolytic uremic syndrome and thrombotic thrombocytopenic purpura?

Anemia is intravascular, and the blood smear shows schistocytes, helmet cells, fragmented red cells. LDH and reticulocyte count will be elevated and the haptoglobin decreased.

29

What is the treatment of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome?

Plasmapheresis. Dipyridamole may preventing platelet aggregation in TTP. Antibiotics contraindicated in HUS; organism releases toxins. Administering platelets will worsen CNS and renal.

30

What is Alport syndrome?

Glomerular disease with anterior lenti conus (lens protrusion), and sensorineural hearing loss.