Baylor University Medical Center Proceedings January 2014, Volume 27, Number 1

was treated empirically for Pneumocystis jiroveci pneumonia, and treatment for disseminated histoplasmosis and mycobacterium avium complex was reinitiated. Approximately 24 hours following admission, a repeat chest radiograph demonstrated increasing bilateral opacities. The work of breathing had increased, and 4 L/min supplemental oxygen via nasal cannula was initiated. Arterial blood gas revealed a worsening pO2 of 59 mm Hg. His oxygen requirement further increased to 10 L/min via nonrebreather mask to achieve a saturation of 92%, and he was electively intubated. Following transfer to the intensive care unit, the patient’s highly active antiretroviral therapy (HAART) regimen was altered from efavirenz/emtricitabine/tenofovir back to lopinavir/ ritonavir/abacavir/lamivudine. Pathologic specimens from a previous lymph node biopsy revealed positive HHV-8 staining, consistent with KS. Bronchoscopy, bone marrow biopsy, and lesion biopsy were nondiagnostic, with negative staining for Pneumocystis jiroveci. All empiric antibiotic and antifungal treatments were stopped after multiple negative diagnostic studies. He was started on doxorubicin treatment once every 3 weeks. Ventilator weaning was initiated and a tracheostomy placed. The patient demonstrated subjective and objective responsiveness to the doxorubicin treatment, with improved pulmonary inﬁltrates, decreased diﬀuse lymphadenopathy, and clinical improvement. After 20 days he was discharged to his home without supplemental oxygen. Our patient clearly presented with a disseminated or metastatic form of KS, with initial involvement of the lymphatics and subsequent development of rapidly progressive, fulminant pulmonary disease. While metastatic KS represents a less common disease variant, a detailed search revealed only one description of 11 patients with KS in whom cutaneous involvement was absent. In 2005, Stebbing and colleagues were the ﬁrst to describe an HIV-infected cohort of 5932 patients, out of which only 11 were identiﬁed as having noncutaneous KS (9). Our patient presented with AIDS clinical trial group stage T1I1S1 disease, which was noted in just 3 of the 11 patients described by Stebbing and associates, possibly indicating an infrequent presentation of an already rare disease variant. Contributing to diagnostic diﬃculty is H

Baylor University Medical Center Proceedings January 2014, Volume 27, Number 1 | Page 34