Abstract Kaposi sarcoma (KS) is an angioproliferative malignancy of endothelial cells that occurs in immunocompromised individuals infected with human herpesvirus-8 (HHV-8). Pulmonary involvement is uncommon, and the diagnostic and therapeutic challenges associated with this manifestation are demonstrated in the following case. A 32-year-old man with asthma, methamphetamine use, and psychiatric illness presented after a new human immunodeficiency virus (HIV) diagnosis. He had odynophagia, cough, nausea, and skin lesions. Labs showed a high viral load and a CD4 count below 20. He was started on antiretrovirals and prophylaxis for opportunistic infections. A month later, he developed hemoptysis, bloody diarrhea, and new oral lesions. Imaging showed lung nodules; biopsies confirmed disseminated KS. He was treated with liposomal doxorubicin but required repeated hospitalizations for hypoxic respiratory failure over the next eight months. During his final hospitalization, there was concern for severe multilobular pneumonia. Bronchoalveolar lavage revealed no evidence of Pneumocystis jirovecii, viral inclusions, or malignant cells, but did identify Candida. Inflammatory markers, including interleukin 6 (IL-6) and C-reactive protein (CRP), were elevated. Although glucocorticoids are contraindicated in KS, the patient's symptoms worsened as they were tapered. Progressive disease prompted a change in chemotherapy to paclitaxel, with the addition of rituximab. Despite aggressive treatment, the patient died shortly thereafter. Kaposi sarcoma cytokine inflammatory syndrome (KICS) was proposed as a unifying diagnosis to explain the clinical presentation. This condition can mimic severe sepsis, yet antibiotics provide no benefit. Immunomodulatory therapy, such as tocilizumab, is the primary treatment approach, but this patient did not receive it. It is possible that earlier recognition and targeted therapy for KICS, including agents like tocilizumab, could have altered his clinical trajectory. KICS is particularly challenging to manage because immunosuppressive or cytotoxic therapies intended to control the inflammatory response can further compromise immunity in patients who are already profoundly immunocompromised. This increases the risk of opportunistic infections and sepsis, complicating management and potentially worsening outcomes. Overall, this case underscores the complexity of diagnosing and treating KICS, as it closely mimics sepsis and requires a high index of suspicion for timely and appropriate intervention. This abstract is funded by: None
Rudmann et al. (Fri,) studied this question.