Journal of the Louisiana State Medical Society
Intravenous Sodium Thiosulfate For Treatment of Calcinosis in Rheumatic Disease B.P. Miller Earl K. Long Medical Center, Baton Rouge Introduction: Calcinosis, or dystrophic calcification, is a poorly understood and debilitating condition that is a commonmanifestation in connective tissue diseases such as scleroderma and systemic lupus erythematosus. Currently, many treatment modalities have been tried with minimal success. While intravenous sodium thiosulfate (STS) is used to treat calciphylaxis and cyanide toxicity, data is not read- ily available regarding its use in calcinosis. Due to similar proposed mechanisms of disease, the administration of in- travenous STS may have a role in the treatment of calcinosis in patients with rheumatic disease. Case: A 63-year-old woman with a history of limited scleroderma with calcinosis and Raynaud’s syndrome, pre- sented to clinic after being treated by an outside physician. For several years, she had recurrent calcinosis deposits on her hands, wrists, knees, and elbows, which caused chronic pain and limited range of motion. These symptoms per- sisted, despite being treated with appropriate scleroderma therapy. Her calcinosis was treatedwith corticosteroids, col- chicine, calcium channel blockers, and surgerywithminimal change in pain level and functional status. After discussion regarding risks and benefits of intravenous thiosulfate, a 25-gram infusion of thiosulfate over one hour, per week was initiated. Despite missing several infusion sessions throughout the year, and receiving 12.5 gram infusions at times, she reported significant improvement in pain and range of motion in her extremities. Overall, the infusions were tolerated well, despite one episode of blurry vision and elevated temperature. Discussion: This case illustrates the potential use of intravenous STS in patients suffering from calcinosis. Cal- cinosis affects approximately 25% of patients with sclero- derma andmay lead to severe disability, pain, and infection. While both mechanisms of disease are poorly understood, calcinosis is pathologically different than calciphylaxis. However, the mechanism of action of STS may also benefit calcinosis. One proposed mechanism of action involves the formation of water soluble calcium-thiosulfate complexes that dissolve existing insoluble calcium salts embedded in tissue. STS may also improve endothelial function through its antioxidant and anti-inflammatory properties. Our case highlights the need for further studies of intravenous STS as a potential treatment option for calcinosis. Dilated Cardiomyopathy Secondary to Mitochondrial Encephalopathy With Lactic Acidosis and Stroke-Like Episodes A. Graebert and B. Lo LSU- Health Sciences Center, New Orleans Introduction: Mitochondrial encephalopathywith lactic acidosis and stroke-like episodes (MELAS) is a maternally
inherited mitochondrial syndrome typically diagnosed in childhood or early teenage years. The stroke-like episodes typically present in a relapsing-remitting manner with gradual neurological decline leading to dementia. Case: A 32-year-old African-American male with a diagnosis of MELAS, confirmed by muscle biopsy eight years prior, presented with a two-month history of short- ness of breath, dyspnea on exertion, and lower extremity edema. The patient was afebrile with a blood pressure of 108/90, heart rate of 103, respiratory rate of 20, and a room air oxygen saturation of 100%. Cardiac exam revealed a III/VI holosystolic murmur heard best at the apex and an S3. Lung exam was unremarkable. He had bilateral lower extremity pitting edema extending to his hips. Labora- tory studies showed a WBC count of 5.7 K/uL with 71% neutrophils. His complete metabolic profile revealed a HCO3 18 mmol/L, BUN 38 mg/dL, creatinine 1.67 mg/ dL, bilirubin 3 mg/dL, AST 92 U/L, ALP 213 U/L, and ALT 100 U/L. BNP was greatly elevated at >5,000 pg/mL. Initial troponin was elevated at 0.14 ng/mL and peaked at 0.17 ng/mL. Lactic acid was also elevated at 3.9 mmol/L. Urinalysis did not contain any protein. Urine electrolytes revealed a fractional excretion of sodium of 6.7%; no urine eosinophils were present. EKG demonstrated normal sinus rhythm with evidence of left atrial enlargement, and chest X-ray revealed mildly increased pulmonary vasculature. The patient was found to have four-chamber enlargement, moderate to severe tricuspid regurgitation, and an ejection fraction <20% by echocardiogram. Diuresis with IV furose- mide provided improvement in his respiratory status and edema; however, he continued to be oxygen-dependent at the time of discharge. Discussion: MELAS is a rare mitochondrial disorder that not only affects the nervous system, but can also have cardiac, renal, and endocrine manifestations, as were seen in this patient with a dilated cardiomyopathy, renal insuf- ficiency, and diabetes mellitus. A hypertrophic cardiomy- opathy is more commonly associated with MELAS, but a dilated cardiomyopathy has also been described.
Acquired Hemophilia A: The AHA Moment S. Fulton, S. Boda, and S. Greenberg LSU-Health Sciences Center, Shreveport
Introduction: Recognition of Acquired Hemophilia A (AHA) without personal or family history of bleeding is difficult. The infrequency of this disease warrants aware- ness of its relatively complicated diagnostic ladder and laboratory findings. Case: A 47-year-old African-American male with past medical history of AIDS and anaplastic large cell lymphoma stage II in remission after chemo and radiation therapy in 1998 presented to the emergency department for continu- ous hemorrhaging for four hours after tongue biopsy. The biopsy site was sutured, and he was discharged home. Two months later, the patient presented to the ED with large, painful, erythematous areas of swelling to the left antecubital
88 J La State Med Soc VOL 166 March/April 2014
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