Connective tissue diseases.

Question 1

Associated findings with ANA-negative lupus

Answer 1

Arthritis, Raynaud’s, subacute cutaneous lupus
Ro (anti-SS-A) Ab +, ANA -
Risk of neonatal lupus in infants of affected women

Question 2

Clinical findings associated with neonatal lupus

Answer 2

Skin lesions
Cardiac abnormalities (AV block, transposition of the great vessels)
Valvular and septal defects

Question 3

Most prominent finding in most patients with SLE

Answer 3

Fatigue – often sign of impending exacerbation

Question 4

Most common cardiac finding in SLE

Answer 4

Pericarditis

Question 5

Hematoxylin bodies are found in what pathology?

Answer 5

Libman Sacks Endocarditis – only difference between this and nonbacterial thrombotic endocarditis. Bodies are found in areas of focal necrosis.

Question 6

Which Abs are peresent in drug-induced lupus?

Answer 6

Anti-histone Abs. Its sensitive but not specific for drug-induced lupus.

Question 7

P-ANCA is positive in what condition?

Answer 7

Polyarteritis nodosa

Question 8

Some commonly implicated agents in drug-induced lupus

Answer 8

Hydralazine, Procainamide, Isoniazid, Chlorpromazine, methyldopa, and quinidine.

Question 9

Best long term tx for SLE

Answer 9

antimalarials like hydroxychloroquine for constitutional, cutaneous, and articular manifestations. Is continued as preventative measure even after resolution of sx but annual eye exam is needed due to retinal toxicity.

Question 10

How do you treat SLE glomerulonephritis?

Answer 10

Cytotoxic agents such as cyclophosphamide

Question 11

Mechanisms of anemia in SLE

Answer 11

Anemia of chronic disease, renal insufficiency from glomerulonephritis, iron deficiency anemia (GI loss due to meds like NSAIDs), autoimmune hemolytic anemia. Can also be caused by medications, hypersplenism, microangiopathic hemolytic anemia, and aplastic anemia.

Question 12

Mechanisms of leukopenia in SLE

Answer 12

Autoimmune mediated destruction. Uncommonly due to medications, hypersplenism, or bone marrow dysfunction.

Question 13

Mechanisms of thrombocytopenia in SLE

Answer 13

Immune mediated destruction. Uncommonly due to medications or increased consumption due to thrombotic microangiopathy (TTP)

Question 14

Pathophys of scleroderma renal crisis

Answer 14

Occurs in up to 20% of pts with diffuse cutaneous systemic sclerosis. Increased vascular permeability, activation of the coat cascade and increased renin secretion causes pts to develop sudden onset of renal failure and malignant HTN. UA can be normal or show mild proteinuria. Peripheral blood smear can show microangiopathic hemolytic anemia with fragmented red blood cells and thrombocytopenia

Question 15

SE of hydroxychlorquine

Answer 15

retinopathy. need eye exams q 6 months

Question 16

Which form of PM/DM have the worst long term prognosis?

Answer 16

Those with antisynthetase Ab are often associated with ILD, other extra muscular mx, and a worse long term prognosis due to the related lung disease.

Question 17

Which agent is preferred in addition to glucocorticoids in patients who have ILD associated with myositis?

Answer 17

Azathioprine. Also preferred if pt has underlying liver dz or unwilling to abstain from alcohol.

Question 18

SE of azathioprine

Answer 18

Systemic flu like run associated with fever and GI complaints. Other side effects includee bone marrow suppression, pancreatitis, and liver toxicity. Long term may include increased risk of malignancy.