This year’s Curbside Consult: Ask the Professors session tackled three tricky treatment challenges for various aspects of lupus, including resistant membranous lupus nephritis, small-fiber neuropathies, and hair loss.
“There is hope on the horizon for therapies for patients with refractory nephrotic syndrome or proteinuria,” said Ellen M. Ginzler, MD, MPH, Distinguished Teaching Professor of Medicine and Chief of Rheumatology at SUNY Downstate Medical Center.
Dr. Ginzler reviewed a case of a patient with Pure Class V Membranous Lupus Nephropathy (MLN) and the steps toward diagnosis and treatment. While MLN accounts for only 10 percent to 20 percent of cases of lupus nephritis and has a better prognosis than the proliferative classes of lupus nephritis, it is still associated with significant morbidity, Dr. Ginzler said.
“This is not a trivial problem,” she said. “It’s important to think about what the consequences may be even when you select initial treatment.”
The guidelines for initial therapy of MLN from both the ACR and EULAR call for initial treatment with prednisone and mycophenolate mofetil (MMF), with possible adjunct treatment of ACE inhibitors, angiotensin II receptor blockers, and/or statins.
“There are many places that will choose intravenous cyclophosphamide as the initial treatment,” she said. “There’s very little data to compare IV cyclophosphamide to MMF.” Although, she added, what few studies exist seem to indicate little difference in outcomes.
She also reviewed calcineurin inhibitors for recurrent or refractory MLN and rituximab as rescue therapy, as well as encouraging clinicians to pursue the available clinical trials that are recruiting patients.
The next presentation looked at the clinical spectrum, diagnostic strategies, and treatment approaches for small-fiber neuropathies in rheumatic diseases.
Julius Birnbaum, MD, MHS, Assistant Professor of Medicine at Johns Hopkins, noted that small fiber neuropathies (SFN) are under-recognized in rheumatology, and delayed diagnosis can contribute to disease refractoriness.
If SFN is suspected, Dr. Birnbaum suggests a punch skin biopsy as a highly reliable method of diagnosis. Samples should be taken from two standardized sites (proximal thigh and distal leg) and can identify clinico-pathological subsets that highlight mechanisms.
Treatment of SFN can be challenging because it requires not only symptomatic therapies but also immunomodulatory therapies.
“The treatment goal that is used in seizures is treat toward efficacy or toxicity. It is a really important goal here that I think should be embraced,” he said, adding that SLN treatment also calls for rational use of polysymptomatic agents.
He suggested trying underutilized topical agents such as capsaicin, which activates and then desensitizes the TRPV1 receptor, or even lidocaine cream.
“Be bold and creative in trying your polysymptomatic approaches,” he said, noting that patients will appreciate if you provide an “optimistic portrayal of therapeutic nihilism.”
Victoria P. Werth, MD, Chief of Dermatology at the University of Pennsylvania, said most cases of lupus specific alopecia are clinically straightforward.
“If they are not, they may require a skin biopsy of the scalp, or they might require immunofluorescence,” she said. “If it’s confusing, we may need to do clinical-pathological correlation to come up with the best diagnosis in those rare cases that are not clear.”
There are three alopecia conditions in patients with lupus: Lupus specific, lupus nonspecific, and alopecia in lupus patients unrelated to lupus. While lupus specific alopecia is associated with underlying erythema and scale, lupus nonspecific alopecia is non-scarring and is not specific to lupus because it can occur in other conditions.
Treatments include sun avoidance and scalp protection, topical steroids, and hair weaves and pieces.
“We sometimes use topical tacrolimus and pimecrolimus,” she said. “We use antimalarial drugs just like for other forms of skin lupus and these can be very effective half the time, and when they aren’t we move on to immunosuppressants. For various severe cases, we may move on to thalidomide or lenalidomide off-label.”
She noted there are very few trials in this area but for resistant and refractory patients there are a lot of new drugs in the pipeline.