Complications
Regression of cutaneous KS lesions may result in hyperpigmentation.[47]
Lymphoedema may precede or accompany the KS lesions, especially on the lower extremities, face, and genitalia. Extensive facial lymphoedema associated with KS is a poor prognostic sign and warrants the use of systemic therapy.[81]
Traumatised mucocutaneous lesions may bleed and present with upper or lower gastrointestinal bleeding as well as haemoptysis. Although cutaneous lesions bleed infrequently following biopsy, mucosal lesions in the respiratory tract may result in frank bleeding.[82]
Ulceration of overlying epidermis or mucosa may predispose to infection.
People with HIV-associated KS who are treated with antiretroviral therapy (ART) can develop immune reconstitution inflammatory syndrome (IRIS); the incidence has been reported to be 3% to 39%.[24][83][84] KS-associated IRIS typically presents with new or enlarged lesions, or worsening lymphadenopathy or oedema within 3 months of starting ART. It may be difficult to distinguish clinically from progressive disease. Risk may be increased for those with low baseline CD4+ T-cell counts and high HIV viral loads.[2][85] Treatment includes systemic therapy and symptomatic management; ART should not be interrupted in patients with IRIS except in life-threatening cases. The decision to use corticosteroids should be made in collaboration with an experienced HIV clinician, because of the potential to exacerbate KS and increased risk of mortality.[85][86][87] Thalidomide may be considered in patients with progression after initial systemic therapies. Patients taking thalidomide must follow strict contraceptive practices as thalidomide is teratogenic. In the US, patients and medical staff must adhere to a Risk Evaluation and Mitigation Strategy (REMS) to ensure that the benefits of the medication outweigh its risks.
In general, KS lesions are painless. However, traumatised lesions on the feet may affect ambulation and painful intra-oral lesions may affect mastication, speech, or swallowing.
Multicentric Castleman's disease (MCD) and KS inflammatory cytokine syndrome (KICS) are rare, life-threatening conditions that can affect patients with HIV-associated KS.[84][88][89] Unexplained fever should prompt work-up for KICS and MCD in patients with HIV-associated KS. Clinical features of KICS and MCD are similar; excisional biopsy of lymphadenopathy is needed to distinguish between them.[2] Rituximab is used as initial treatment for active MCD (without organ failure), but may cause flares of KS.[42] Optimal management of KICS is unknown; however, rituximab has been used in some cases.[89] Immune checkpoint inhibitors should not be used in patients with MCD or KICS because of the risk of exacerbation.[2]
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