INTRODUCTION: Kaposi's sarcoma (KS) is the most common neoplasm associated with human immunodeficiency virus (HIV) infection. Pulmonary involvement is well described and associated with poor prognosis. Pulmonary KS (PKS) without concomitant mucocutaneous disease is considered rare. We report a case of disseminated KS in an individual with advanced adult immune deficiency syndrome (AIDS) whose initial presenting symptoms comprised of dyspnea and cough, without dermatological manifestations of KS.
CASE PRESENTATION: A 40-year-old homosexual Caucasian man with AIDS (CD 4 cell count, 60 cells/uL; HIV RNA, >100,000 copies per milliliter) was admitted with progressive dyspnea and cough of 2-week duration. He reported associated 6-month history of night sweats and 35-pound weight loss. There was no travel history or sick contacts. His past medical history was notable for oral candidiasis, genital herpes, and condylomata. He was not receiving highly active antiretroviral therapy (HAART).On presentation, the patient was afebrile with a respiratory rate of 16 breaths per minute and an oxygen saturation of 92% on room air. Physical examination demonstrated bilateral inspiratory crackles, generalized lymphadenopathy and hepatosplenomegaly, without skin lesions. Chest radiography (CXR) showed right airspace infiltrates. The patient responded well to empiric coverage for bacterial pneumonia and Pneumocystis jiroveci pneumonia (PJP) therapy, including oral prednisone. No endobronchial lesions were visualized on bronchoscopy. Cultures for infectious etiologies obtained at bronchoscopy were negative, including PJP cytology stains. PJP therapy was stepped down to prophylactic doses and prednisone was tapered. The patient noticed recurrence of his symptoms coinciding with decreasing prednisone dosage. He was readmitted 12 days post discharge. Good clinical response was again achieved with empiric prednisone-containing PJP treatment and bacterial pneumonia therapy; therefore, a bronchoscopy was not performed. No skin lesions were identified during this admission. One week after discharge, the patient presented with a 2-day history of hemoptysis. He again reported worsening of his dyspnea after completion of PJP therapy and prednisone taper. Violaceous lesions consistent with KS were now present inside the oral cavity and on the face. CXR showed worsening mixed airspace and interstitial infiltrates bilaterally. Hypoxic respiratory failure and multi-system organ failure led to the patient's demise, despite aggressive medical therapy. Autopsy confirmed the underlying cause of death in this patient to be AIDS with disseminated KS involving skin, lungs, liver, spleen and lymph nodes.
DISCUSSIONS: PKS is known to be associated with particularly poor prognosis; however, PKS in the absence of skin lesions is rare. A clinical diagnosis of PKS is often elusive because of its non-specific respiratory symptoms and radiological findings that can mimic numerous other infectious and neoplastic processes seen with AIDS. We report a case of disseminated PKS in an individual with advanced AIDS not on HAART who presented with cough and dyspnea, in the absence of cutaneous KS. His favorable responses to empiric antimicrobial therapy confounded his diagnosis. A temporal relationship between clinical deterioration and tapering of oral prednisone was observed in this case. His symptomatic improvement might be attributable to the treatment of occult concomitant opportunistic infections, in addition to the anti-inflammatory effects of corticosteroids and reduction of airway inflammation. However, glucocorticoids have been previously shown to activate human herpes virus-8 (HHV-8) and induce KS spindle cell proliferation. The exposure to high dose prednisone may have in fact accelerated disease progression in our patient.
CONCLUSION: In individuals with AIDS and respiratory symptoms, the diagnosis of PKS should be included in the differential, despite the absence of cutaneous KS.
DISCLOSURE: Julie Chou, No Financial Disclosure Information; No Product/Research Disclosure Information