A 24-year-old African American male with three week history of newly diagnosed HIV infection, presented to the emergency department (ED) with 1 month history of pleuritic chest pain, shortness of breath and nonproductive cough. He also experienced a 15 pound weight loss over the past 3 months with decrease in appetite. He was only sexually active with women. The patient had missed his first scheduled follow up visit and so he had not been initiated on ART.
Initial physical exam in the ED, showed the following: blood pressure of 129/64 mmHg, heart rate of 117 beats/min, temperature of 98.1 F, respiratory rate of 17 breaths/min and oxygen saturation of 100% while on room air. He was alert and oriented and not in distress. He was noted to have non-tender right sided cervical lymphadenopathy and bilateral rhonchi at lung bases. There was no skin breakdown or rash seen on inspection. Laboratory results were significant for WBC 3200/mm with 13% lymphocytes, hemoglobin 11.6 g/dL, LDH 272 U/L, creatinine 1.9 mg/dL, CD4 count 54/mm, and HIV viral load 334,991 copies/mL. Chest radiograph (CXR) showed coarse bilateral lower lung interstitial infiltrates and mild perihilar infiltrates (Fig. 1).
He was admitted to the hospital for suspected HIV related pulmonary infection and empirically started on trimethoprim/sulfamethoxazole and azithromycin. Workup was negative including evaluation for Mycobacterium tuberculosis, Legionella pneumophila, Histoplasma capsulatum, Cryptococcus neoformans, and viral respiratory panel including Influenza virus. On day 3 of hospital admission, physical exam revealed a new papular dark violaceous skin rash under his left eye, and lesions on his left shoulder, bilateral arms and torso. The papules were suspicious for KS and a left shoulder skin biopsy was performed. The biopsy showed a vascular tumor positive for HHV-8 immunostain confirming the diagnosis of KS. Meanwhile, a bronchoalveolar lavage (BAL) was done and silver stain was negative for Pneumocystis jirovecii pneumonia (PJP). Nine days after admission, his creatinine levels progressively worsened from 1.86 on initial presentation to 2.70. It was determined that trimethoprim/sulfamethoxazole was contributing to his acute kidney injury and he was switched to atovaquone for PJP prophylaxis.
High resolution chest CT (Fig. 2) was performed which revealed diffuse irregular opacities of varying appearance with linear and nodular features, mixed confluent and nodular soft tissue and interlobular septal thickening. Bronchoscopy with BAL was repeated. No bronchial lesions were visualized and, again, the pathology and cultures were negative. During the course of his hospital stay, patient continued to develop fevers as high as 102 degrees Fahrenheit with multiple episodes of diarrhea, tachypnea, tachycardia, and hypoxemia. However, blood cultures and Clostridium difficile toxin were negative. His lung auscultatory findings worsened and diffuse bilateral crackles and rhonchi were audible. CXR showed worsening pleural effusions bilaterally.
Video assisted thoracoscopic surgery (VATS) was done which showed multiple diffuse hemorrhagic lesions in the right upper, middle, and lower lung lobes with a suspicion for KS. The right middle lobe lung biopsy revealed multifocal perivascular atypical spindle cell proliferation staining strongly positive for HHV-8 Latency Associated Nuclear Antigen. (Fig. 3) He was diagnosed with AIDS related KS and was started on ART therapy. Patient was transferred to an outside hospital after 33 days of hospitalization for chemotherapy. He received 6 cycles of doxorubicin and his last cycle was 4 months after initial diagnosis. He was seen at an infectious disease outpatient clinic 3 months after diagnosis and was doing well with a CD4 count of 211/mm and a viral load of 431 copies/mL.