Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:57

Polymyositis and Related Disorders Porphyria, Acute 1193

■Respiratory failure & congestive heart failure are rare. 50–75% of pts

will go into complete remission.

■If pt fails to respond to treatment:

➣Add another drug

➣Consider steroid myopathy

➣Question the accuracy of the diagnosis

➣Question if dealing w/ known unresponsive disease-types-

associated-malignancy IBM-anti-SRP or anti-synthetase syn-

drome

➣Consider using cyclosporine, cyclophosphamide, chlorambucil

or IVIg

PORPHYRIA, ACUTE

D. MONTGOMERY BISSELL, MD

history & physical

History

■Young adult, female more often than male, Caucasian more than

African, Indian or Asian; very rare before puberty; declining inci-

dence after age 50.

■Recent caloric restriction, due to illness or strict diet regimen.

■Use of porphyria-inducing medication, e.g. barbiturates, phenytoin,

valproate, sulfa drugs, estrogens.

■Constipation: common, often chronic

■History of recurrent pain attacks; negative exploratory surgery; pre-

menstrual pain attacks

■Steadily increasing nausea and pain (abdomen, back or extremities)

over days (not hours).

■Dark urine, depending on the type of porphyria; in acute intermittent

porphyria (AIP) urine color may be normal.

■Mental status change: often subtle (e.g., hysterical affect); seizures

can occur; florid psychosis is unusual.

■Family history: often negative. Relatives with similar attacks of pain

or a devastating, undiagnosed neurological condition.

Signs & Symptoms

■Tachycardia common; fever usually absent

■On abdominal exam, reduced bowel sounds (suggestive of ileus);

diffuse or focal tenderness but less than degree of pain would suggest.

No rebound tenderness.