Case Presentation: A 28-year-old man from Central America with a history of newly diagnosed human immunodeficiency virus (CD4 119 cells/mm^3, CD4 8%, viral load 61,000 copies/mL) presented with two months of progressive shortness of breath. Physical exam was remarkable for anasarca, diffuse cutaneous purpuric plaques as well as bilateral respiratory crackles. Chest x-ray revealed bilateral pleural effusions. Skin biopsy confirmed cutaneous Kaposi sarcoma; bronchoscopy and magnetic resonance imaging were suggestive of pulmonary and hepatic metastases (Figure 1). Anti-retroviral therapy was re-initiated and docetaxel was started. He then developed progressive distributive shock requiring aggressive hemodynamic support, as well as respiratory failure necessitating repeated thoracenteses and chest tube placement, initiation of bi-level positive airway pressure, and eventually intubation. Repeated infectious workup was negative. Upon further goals of care discussions, the family wished the patient to not be resuscitated in light of any further decompensation. He eventually passed away due to worsened distributive shock.
Discussion: We describe a rare case of severe pulmonary Kaposi sarcoma-immune reconstitution inflammatory syndrome despite therapy. Kaposi sarcoma is defining of acquired immunodeficiency syndrome. Initiating antiretroviral therapy in these patients can sometimes result in a systemic immune response. This phenomenon is known as an immune reconstitution inflammatory syndrome and is typically associated with tuberculosis, cryptococcal meningitis, and cytomegalovirus retinitis. While rare, patients with extensive tumor burden from Kaposi sarcoma can experience an immune-related inflammatory reconstitution that can clinically manifest as undifferentiated shock. This unusual condition is defined as ≥2 of the following: an abrupt increase in number of biopsy-proven cutaneous lesions, appearance or exacerbation of lung-opacities or lymphedema, concomitant increase in CD4 cell-count ≥50 cells/mm^3 and a decrease of >1 log in viral-load once started on therapy. While initiation of antiretroviral and chemotherapy has been associated with positive outcomes, this condition may be accompanied by respiratory failure and thus has a high degree of mortality as demonstrated in our case. A retrospective study demonstrated an incidence of immune reconstitution syndrome in 6% of individuals with Kaposi sarcoma receiving interventions with improvement in mortality.
Conclusions: In severe Kaposi sarcoma, an extensive goals of care conversation should be initiated early. This case describes the rare but clinically significant phenomenon of Kaposi sarcoma immune reconstitution syndrome and highlights the possibility of paradoxical decompensation despite therapy.