Rituximab may also be a therapeutic option for adults with Sjögren’s syndrome who manifest any or all of the following: vasculitis, with or without cryoglobulinemia; severe parotid swelling; inflammatory arthritis; pulmonary disease; and peripheral neuropathy, especially mononeuritis multiplex.
“There probably is some discrepancy or dichotomy between what people may have gleaned from published conclusions of trials of rituximab for Sjögren’s and what the topic review group and the committee and consensus panel recommended,” Dr. Carsons says. “One has to dissect the data and examine secondary outcome measures and time points in order to come to an understanding that there may well be a benefit to the use of agents that target B cells in treating this disorder.
“Although no randomized clinical trial of rituximab in Sjögren’s syndrome has reached its primary endpoint to date, B cell depletion appears to show promise for both glandular and extraglandular manifestations of primary Sjögren’s syndrome, including unresponsive inflammatory arthritis, as well as cryoglobulinemic vasculitis, interstitial lung disease and neuropathies, particularly mononeuritis multiplex,” he says.
Management of Fatigue
Fatigue is one of the most difficult management dilemmas in Sjögren’s syndrome, according to the authors, and the causes of fatigue are numerous. A comprehensive diagnostic evaluation should be conducted to exclude anemia, hypothyroidism, sedating medications and sleep disorders. Patients should be given advice about the value of exercise, which is the only strong recommendation for fatigue in patients with Sjögren’s.
The guideline indicates that hydroxychloroquine (HCQ) may be considered for treatment of fatigue in selected situations, such as presence of inflammatory markers, joint pain or rash. HCQ is the most widely prescribed treatment to manage fatigue in Sjögren’s in the U.S., but that practice is largely based on uncontrolled studies and clinical experience; thus, the recommendation was rated as weak and the overall quality of evidence was given a very low rating by the committee.
“Additional studies with different patient selection parameters, longer duration of therapy and alternate outcomes measures are needed before concluding that use of HCQ should be precluded in this setting,” according to the guidelines.
The guidelines do not recommend the use of biologics, such as TNF inhibitors, rituximab or newer agents, or dehydroepiandrosterone (DHEA) for fatigue alone.
Musculoskeletal Pain
Recommendations related to the management of inflammatory joint and musculoskeletal pain are represented by a hierarchical decision tree or sequential approach to therapy. Recommendations for agents that have similar efficacy and safety profiles are grouped together so practitioners can choose treatments based on their own experience and the specific patient circumstances.
