Thank you to Hannah and Mike for presenting a very interesting case of a middle-aged woman with advanced HIV (CD4 104) who presented after a fall and was found to have sepsis from pyelonephritis. During her infectious workup, she was noted to have several vascular skin lesions and a chest x-ray revealing multifocal pulmonary nodules. Ultimately, she underwent bronchoscopy where she was diagnosed with pulmonary Kaposi sarcoma (KS), in addition to cutaneous KS on her soft palate and skin. Though the Kaposi sarcoma was an incidental finding to her presentation of sepsis, the case was an important reminder that patients with severe immunosuppression can often have multiple diagnoses. It is particularly helpful to involve the infectious disease consult service in patients with very low CD4 to assist in diagnostic workup when you are concerned about the possibility of an opportunistic infection.
A few learning points on pulmonary Kaposi sarcoma:
- Typically only seen in patients with low CD4 (<150) and elevated viral load
- Pulmonary KS usually co-occurs with other mucocutaneous lesions, though up to 15% of patients with pulmonary KS will lack mucocutaneous involvement
- Pattern of involvement is usually parenchymal (dyspnea, hypoxia, dry cough, pleural effusion) or endobronchial (intractable cough, hemoptysis, wheezing, or upper airway obstruction – though can also be asymptomatic)
- Rapid progression of pulmonary KS can occur in the setting of systemic steroids – have a high degree of suspicion for pulmonary KS when patients develop rapid onset of respiratory symptoms following steroid initiation
- Diagnosis is usually made either clinically in the setting of clear mucocutaneous KS lesions, or on direct visualization on bronchoscopy. Endobronchial biopsies are generally not done due to concern for pulmonary hemorrhage with these highly vascular lesions
- Differential diagnosis is broad but includes bartonella (bacillary angiomatosis lesions are similar in appearance to KS, though BA is generally more raised), TB, fungal infections, pulmonary lymphoma, multicentric Castleman’s disease and lung cancer