Cryoglobulinemic vasculitis

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70 year old male with cryoglobulinemic vasculitis, multiple ulcers to the feet and fingers, in various stages of tissue necrosis. The ulcers are quite extensive and he is at risk for amputation. His pain level is high, 7-10/10. He has responded poorly on multiple immunosuppressants, including plasmapheresis. His was referred for hyperbaric oxygen therapy. His insurance is Medicare with supplement. He is not a diabetic, and does not have macrovascular disease or osteomyelitis.

I advised him that his condition is not an HBO covered CMS benefit. Although there are case reports of patients showing improvement with treatment, I am not aware of high quality scientific studies regarding HBOT benefiting his condition. I wanted to make sure that both these statements are correct and if anything else can be offered beyond local wound care.
Appreciated.

Jan 25, 2024 by Bill Khoury,

1 replies

Elaine Horibe Song
MD, PhD, MBA

Hi Dr Khoury,

Thank you for sharing this case. Other colleagues might have additional input; here are some thoughts by Dr Charash, Cathy Milne, Jeff Mize, Tiff Hamm and me:

Yes, it is correct that HBOT as an adjuvant modality for vasculitis-induced non-healing skin ulcers are not covered by CMS in the outpatient setting.
Aside from case reports/series and retrospective studies (see below), there haven't been any RCTs or high quality studies on the effectiveness of adjunctive HBOT for vasculitis-induced non-healing skin ulcers. According to this case series review, there was some evidence that HBOT may improve the healing rate of wounds by increasing nitric oxide (NO) levels and the number of endothelial progenitor cells in the wounds, and that HBOT may also improve pain in these ulcers. Dr Charash also shared his personal experience in successfully treating a patient with digital ulcers due to Raynaud's disease with HBOT.
If the patient is willing/has the means to, and assuming no contraindications to HBOT, one option is to have him sign an ABN through Medicare, then try an in-chamber TCOM and assess whether there is more or less vasoconstriction/pain with HBOT. If good response, adjunctive HBOT under problem wound support protocol (2.0 ATA, 90 min of oxygen breathing, as per UHMS indications manual) might be considered. As mentioned before, this is not a CMS covered indication though.
As you pointed out, it'd be important to work with rheumatology, heme/onc and see if there are any other main interventions that address his underlying condition. See excerpt below from this review article on management of refractory cryoglobulinemic vasculitis:

The goals of therapy for mixed cryoglobulinemia include immunoglobulin level reduction and antigen elimination.
Cryoglobulinemic vasculitis (CryoVas) not associated with HCV infection should be treated according to treatment recommendations for small-vessel vasculitides.
CryoVas associated with chronic HCV infection (90% of cases of mixed cryoglobulinemia) should be treated with antivirals along with immunosuppressive drugs, with or without plasmapheresis, depending on disease severity and organ involvement. Patients who do not respond to first-line therapy may achieve remission when treatment with rituximab is started as second-line therapy. In HCV-related CryoVas, antiviral therapy should be given along with rituximab in order to achieve complete or partial remission. Moreover, rituximab has proven to be a glucocorticoid-sparing medication. Other potential therapies for refractory CryoVas include mycophenolate mofetil and belimumab, while tumor necrosis factor (TNF) inhibitors are not effective.

In addition, regarding main interventions that address his underlying condition, one can go to clinicaltrials.gov and look for active trials. Even if there are no active trials, it might be possible to find centers with professionals who are experienced in managing this condition

Case series/reports

Jan 26, 2024

* Information provided without clinical evaluation and is not intended as a replacement for in-person consultation with a medical professional. The information provided through Curbside Consult is not a substitute for proper training, experience, and exercising of professional judgment. While every effort has been made to ensure the accuracy of the contents, neither the authors nor the Wound Reference, Inc. give any guarantee as to the accuracy of the information contained in them nor accept any liability, with respect to loss, damage, injury or expense arising from any such errors or omissions in the contents of the work.

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