A 43-year-old African-American man with history of diabetes mellitus, chronic systolic heart failure with ejection fraction of 15%, atrial fibrillation and stage III kidney disease HIV (Human immunodeficiency virus) diagnosed in 1994 on antiretroviral therapy (ART). Kaposi sarcoma of the skin and lung diagnosed a year ago with lung wedge biopsy and on chemotherapy, presented with 1-week history of progressive worsening shortness of breath with minimal efforts, pleuritic chest pain and productive cough with ‘penny-sized blood clots’ that were both dark and bright red in color. Furthermore, he reported nocturnal diaphoresis over the same period. Denies any recent exposures or travel outside the USA has been compliant with Trimethoprim/Sulfamethoxazole (TMP/SMX) for Pneumocystis jiroveci Pneumonia (PJP) prophylaxis three times a week for many years. On exam, he was febrile (38.1°C) and hypoxic (SpO2 91% on 4 L/min O2 per nasal cannula). He had bilateral coarse crepitations at bases on posterior chest exam. Blood work showed hemoglobin 7.9 g/dl (down from basal of 12.2 g/dl a week prior). Chest roentgenogram (CXR) showed diffuse bilateral mixed interstitial and alveolar infiltrates with bilateral mild pleural effusions. Giving his clinical presentation and history, CT scan of the chest was done and showed diffuse ground-glass opacity admixed with consolidative opacity in the right upper, middle, and lower lobes and the left upper lobes. This was associated with numerous bilateral, mid and lower lung solid pulmonary nodules with soft tissue infiltration along bronchovascular bundles, these findings represent Kaposi's sarcoma (KS) with diffuse alveolar hemorrhage (Figures 1 and 2). Intravenous antibiotics were administered including azithromycin, piperacillin/tazobactam and vancomycin; TMP/SMX was continued. He had rapidly progressive deterioration of his respiratory status, requiring 100% high-flow oxygen and subsequently airway intubation and mechanical ventilation. Few hours later he had pulseless electrical activity (PEA) cardiac arrest and passed away after 30 min of advanced cardiopulmonary resuscitation efforts.
KS is low grade vascular tumor that has been linked with human herpesvirus 8, also known as the KS-associated herpesvirus (KSHV). Its prevalence and aggressiveness have decreased associated with the widespread use highly active ART. KS initially occurred in ∼6–20% of HIV-infected men and a small number of non-HIV-infected patients from other risk groups.1,2 The clinical presentation of KS varies from indolent skin lesions to extensive visceral disease. Cutaneous lesions are the most common presentation for KS.3
Patients with pulmonary KS can present with dyspnea, fever, cough, hemoptysis or chest pain.4 These symptoms can be attributed to infectious process. Respiratory failure due to diffuse alveolar hemorrhage can occur and considered the most common cause of mortality in patients with pulmonary KS.4 Among patients with known KS who present with respiratory symptoms, up to 50% have parenchymal involvement by KS.5
CXR, CT scanning and nuclear imaging are useful tools in the diagnostic work-up of HIV-infected patients presenting with pulmonary symptoms. The diagnostic evaluation of suspected parenchymal pulmonary KS requires bronchoscopy in most cases. Endobronchial and transbronchial biopsies have a low diagnostic yield.4,6 The most accurate diagnostic test for pulmonary KS is detection of HHV 8 in bronchoalveolar lavage fluid.
Although biological modifiers such as interferon-α have a role early or late in the course of KS, pulmonary involvement usually requires chemotherapy.7,8 Furthermore, ART should be given in most cases.8–10
Conflict of interest: None declared.