A 30-year old African American female with no significant past medical history initially presented to our emergency department with three days of sore throat, dysphagia, fever, fatigue, nausea and vomiting. She denied ear pain, rhinorrhea, shortness of breath or any sick contacts. Her social history was negative for tobacco, alcohol and illicit drug use. She works as a security officer, lives with her family and is sexually active only with her husband. On initial examination she was febrile to 101.9° F, with a heart rate of 100 beats per minute, blood pressure of 143/99 mmHg, respiratory rate of 18 breaths per minute and an oxygen saturation of 99% on room air. Her only pertinent physical examination findings were a mildly erythematous oropharynx without exudates, mildly swollen uvula and right tonsil, bilateral tender swollen sub-mandibular lymph nodes and reduced breath sounds on auscultation of the right lower lung base.She was routinely tested for HIV, ruled out for group A strep, and discharged home with the diagnosis of viral pharyngitis on supportive care.
Following the identification of a presumptive positive rapid HIV screening test with evidence of HIV-1 p24 antigen and a reactive HIV-1 antibody on the multispot HIV 1 / 2 antibody test she was called to return to the ED for counseling regarding a positive HIV test. She reported continuation of her prior symptoms with worsening dysphagia, as well as new complaints of bilateral lower extremity edema to the knees. Initial laboratory testing revealed an elevated serum creatinine (Cr) of 2.2mg/dL (0.7-1.3 mg/dL) up from <1.0mg/dL one-year prior, with an estimated Creatinine clearance (CrCl) of 43.4 ml/min using the modified Cockcroft-Gault equation. She was admitted for further workup. A trial of IV fluid hydration overnight worsened her symptoms and additional labs demonstrated hypoalbuminemia, 4+ proteinuria with 1+ blood, and a urine protein/creatinine ratio of 17mg/ mg (<0.2 mg/mg), consistent with nephrotic syndrome. Her CD4 count was 115 cells/mm3 (500-1500 cells/ mm3) with an HIV viral load of 117,148 copies/ml. Based off negative labs for syphilis, hepatitis panel, ANA, complement C3/C4, and diabetes, findings were felt to be consistent with HIV Associated Nephropathy (HIVAN).
The patient underwent renal biopsy to confirm the diagnosis and was started on abacavir, darunavir, dolutegravir, lamivudine and ritonavir. Pathology results were consistent with HIVAN with tubulointerstitial nephritis and collapsing glomerulonephropathy and electron microscopy showed diffuse epithelial cell injury with effacement of foot processes and segmental collapse of glomerular capillary loops. Her serum Cr peaked at 2.78 on day 7 of her admission. Her serum Cr and urea-nitrogen steadily improved after just one week of HAART therapy leading to a 42% reduction in serum Cr (Figure 1). Additionally, due to her un-resolving dysphagia the patient underwent esophagogastroduodenoscopy, which was unremarkable. However, she subsequently had esophageal manometry, which was consistent with diffuse esophageal spasm for which she was started on diltiazem.
Danaf, MD, Jad Al; Marbach, MD, Jeffrey; Li, MS4, Sharon; and Stewart, MD, Emily
"Nephrotic Syndrome: Is HIV Associated Nephropathy on Your Differential?,"
The Medicine Forum:
Vol. 16, Article 10.
Available at: http://jdc.jefferson.edu/tmf/vol16/iss1/10