Illustration of lungs with tumor and normal growth, representing Kaposi's Sarcoma treatment.

Steroids to the Rescue? A Rare Case of Kaposi's Sarcoma Treatment

"When HIV treatment falters, a dangerous condition called Kaposi's Sarcoma can emerge. But in a unique case, doctors found an unexpected ally: steroids."


Kaposi's Sarcoma (KS) is a type of cancer that develops from cells that line blood and lymph vessels. It's often linked to infection with the human herpesvirus 8 (HHV-8) and is more common in people with weakened immune systems, such as those with HIV. While KS often appears as skin lesions, it can also affect the lungs and other organs.

Typically, treating KS involves a combination of therapies, including addressing the underlying HIV infection with antiretroviral therapy (ART) and using chemotherapy drugs like liposomal doxorubicin. However, in some cases, the immune system can overreact as it recovers, leading to a condition called immune reconstitution inflammatory syndrome (IRIS), which can paradoxically worsen KS symptoms.

Now, doctors share a case of a patient with severe KS affecting his airway. Initial treatments had limited success, but an unexpected intervention – steroids – proved crucial in managing the condition. This case highlights the complexities of treating KS and the potential for unconventional approaches when standard therapies fall short.

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Global Burden and HIV

More than 34,000 Kaposi sarcoma (KS) cases were estimated globally in 2020, and the source attributes all of them to Kaposi sarcoma herpesvirus (KSHV). KS existed in KSHV-endemic regions, notably sub-Saharan Africa, before the HIV epidemic. The HIV epidemic has substantially increased the KS burden.

KS Through the HIV Era

A 1986 historical review described AIDS-related KS as the fourth form of the tumor. In the early years of the HIV epidemic, KS was common among people with advanced HIV. After effective HIV treatment became available in 1996, KS cases fell dramatically, and long-term HIV treatment lowers the risk of KS.

When Standard Treatments Aren't Enough: A Patient's Story

Illustration of lungs with tumor and normal growth, representing Kaposi's Sarcoma treatment.

A man in his 30s with a history of HIV stopped his regular treatments. He developed a range of worrying symptoms, including recurring fever, sore throat, and a hoarse voice. Doctors discovered purplish tumors in his mouth, swollen lymph nodes, and skin lesions. Tests confirmed he had Kaposi's Sarcoma, with widespread disease affecting his lungs and airways.

The patient's condition quickly deteriorated, leading to severe airway obstruction that required him to be placed on a ventilator in the intensive care unit (ICU). Doctors immediately started him on ART to combat the HIV infection and began chemotherapy with liposomal doxorubicin to target the KS tumors.

  • Initial Improvement: The combination of ART and chemotherapy initially helped to control the disease. The patient was eventually able to come off the ventilator and leave the hospital.
  • Unexpected Setback: After discharge, a follow-up scan revealed new areas of KS in his lungs. This raised concerns about IRIS, where the recovering immune system was actually contributing to the problem.
  • A Risky Decision: Due to side effects from previous treatments, doctors were hesitant to use chemotherapy again. Instead, they made the unusual choice to try a short course of steroids to dampen down the immune system's overreaction.
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Evidence Gaps in KS-IRIS

A review reports that multiple targeted therapies for KS have been evaluated in clinical trials in recent years. However, to the review authors’ knowledge, none had been studied in patients with KS-associated immune reconstitution inflammatory syndrome (KS-IRIS). The authors therefore say the therapies’ roles in preventing or treating KS-IRIS cannot be defined.

Steroid-Associated Risks

In a study of people with HIV, glucocorticoid use was associated with higher mortality, with an adjusted odds ratio of 4.719 (95% confidence interval 1.383–16.103; P = 0.0132). Glucocorticoid use was also associated with a shorter time to KS-IRIS (P = 0.03). The study additionally reported a shorter time to death (P = 0.0073) among those using glucocorticoids.

Remarkably, the steroids worked. The patient's lung lesions shrank, and he remained stable for two years without further KS recurrence. This case suggests that in certain situations, steroids can be a valuable tool for managing KS-IRIS when other options are limited.

What Does This Mean for KS Treatment?

This case offers a valuable lesson for doctors treating Kaposi's Sarcoma. While ART and chemotherapy remain the cornerstones of treatment, it highlights the importance of considering IRIS as a potential complication.

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KS: Tumor and Sites of Involvement

A 2024 literature review describes Kaposi sarcoma as a malignant tumor of endothelial cells caused by human herpesvirus type 8. It reports that KS may affect the skin, lymph nodes, or viscera. The review notes that these affected sites are especially relevant in patients with HIV.

Mapping Global KS Trends

A population-based study set out to describe global patterns and population-wide trends in KS burden. It extracted KS incidence and mortality estimates for 185 countries and regions in 2020 from the GLOBOCAN 2020 database. These figures provide a cross-national snapshot for that year.

Corticosteroid Caution

NIH guidance advises avoiding concurrent systemic corticosteroids or other immunosuppressants in patients with KS when possible. If they are used, the guidance recommends close observation. The stated concern is the potential for KS exacerbation.

Disseminated Disease

A 2024 case report describes AIDS-associated disseminated KS as a rare condition with a rapid, progressive, and highly fatal course if left untreated. It identifies the skin and mucous membranes as the primary sites affected. The report attributes the disease to human herpesvirus 8.

The successful use of steroids in this case suggests that they can be a safe and effective option for managing KS-IRIS in carefully selected patients, particularly when other treatments have failed or are not well-tolerated.

Further research is needed to determine the optimal role of steroids in KS treatment and to identify which patients are most likely to benefit from this approach. However, this case offers a glimmer of hope for those facing the challenges of KS and IRIS.

KS-IRIS rates across chemotherapy comparisons

  • 7% versus 21%In a separate trial of mild to moderate KS, KS-IRIS occurred in 7% of participants given immediate etoposide with ART, compared with 21% with ART alone.[1]

How these findings fit the broader KS-IRIS evidence

A review noted that targeted KS therapies had been evaluated in trials but, in its authors’ account, not in patients with KS-IRIS; their role in preventing or treating the syndrome therefore remained undefined.[1]

NIH guidance warns that systemic corticosteroids may exacerbate KS, advising avoidance when possible or close observation if they are used; a separate mechanistic account describes direct and indirect ways glucocorticoids may promote KS growth.[2] [3]

Another reported option for steroid-refractory or steroid-dependent IRIS is thalidomide, described as having anti-tumor necrosis factor activity and activity against KS through antiangiogenic properties.[4]

Questions the KS-IRIS evidence leaves open

  • What role, if any, do targeted KS therapies have in preventing or treating KS-IRIS when their effects in this patient group have not been established?[1]

About this Article -

Written with AI assistance from published research, and reviewed by the Mystum team. See our About page for more information.

Everything You Need To Know

1

What exactly is Kaposi's Sarcoma?

Kaposi's Sarcoma (KS) is a cancer that originates in the cells lining blood and lymph vessels. It's frequently associated with the human herpesvirus 8 (HHV-8) and is more prevalent in individuals with compromised immune systems, such as those with HIV. KS can manifest as skin lesions, but it can also impact the lungs and other internal organs, illustrating its potential severity and the need for effective treatments.

2

How is Kaposi's Sarcoma typically treated?

Typically, the treatment for Kaposi's Sarcoma involves a dual approach: managing the underlying HIV infection with antiretroviral therapy (ART) and directly targeting the KS tumors with chemotherapy drugs like liposomal doxorubicin. ART is essential to rebuild the immune system, while chemotherapy aims to destroy the cancerous cells. However, the immune system's recovery can sometimes lead to immune reconstitution inflammatory syndrome (IRIS), which can worsen KS symptoms, complicating the treatment process.

3

What role did steroids play in this specific case?

In the case mentioned, the doctors used steroids as an unconventional treatment approach for a patient whose Kaposi's Sarcoma had severely affected his airway. Standard treatments such as Antiretroviral Therapy (ART) and chemotherapy using liposomal doxorubicin had initially shown some success. However, a subsequent setback, potentially linked to Immune Reconstitution Inflammatory Syndrome (IRIS), prompted the use of steroids to calm the overactive immune response, demonstrating a shift in strategy when standard therapies were insufficient.

4

What is Immune Reconstitution Inflammatory Syndrome (IRIS) and how does it relate to Kaposi's Sarcoma?

Immune Reconstitution Inflammatory Syndrome (IRIS) occurs when the immune system, recovering from HIV treatment, overreacts and exacerbates conditions like Kaposi's Sarcoma. In the patient's situation, the initial treatments with ART and chemotherapy were effective. However, the follow-up scan revealed new areas of KS in his lungs. The medical team suspected IRIS was contributing to the problem, leading to the consideration of steroids to control the overactive immune response.

5

What is the significance of this case for the treatment of Kaposi's Sarcoma?

The use of steroids in this case highlights an important consideration for doctors treating Kaposi's Sarcoma. While Antiretroviral Therapy (ART) and chemotherapy remain the primary treatments, doctors should consider the potential for Immune Reconstitution Inflammatory Syndrome (IRIS). The successful use of steroids suggests that in specific scenarios, they can serve as a valuable tool in managing KS-IRIS when conventional therapies are limited or have failed. This shows that doctors must tailor their approach to the individual patient's situation and the potential for unconventional treatments.

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