Chest 2021 Feb
Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's.
Related Questions
Would you perform screening for pulmonary hypertension in a patient who has biopsy-proven Sjogren's but has a centromere antibody?
Generally, the risk of pulmonary hypertension in Sjogren's is low - about 2% in a recent study using RHC for diagnosis (Coppi et al., PMID 40058609). There have been no studies linking Raynaud's to pulmonary hypertension risk in Sjogren's, although this is true in systemic sclerosis. So the real que...
What is your approach to patients with severe sicca symptoms and history of B Cell lymphoma?
For your patient with SjD, B-cell lymphoma, and severe sicca; in addition to treating them with HCQ, I would also: Be diligent with treating the sicca sxs proactively to prevent dental loss and vision problems using the previous guidelines (Zero et al., PMID 26762707 and Foulks et al., PMID 25881996...
What is your preferred steroid sparing agent in the treatment of Sjögren associated LIP?
There is unfortunately very limited data comparing the efficacy of various steroid-sparing agents for the treatment of connective tissue disease-related ILD, and no large prospective studies have specifically addressed the ideal approach for patients with underlying Sjogrens. Mycophenolate and azath...
How would you treat inflammatory arthritis in a patient with Sjogren's syndrome and ILD (on mycophenolate) that is not responsive to hydroxychloroquine?
Fortunately, the Sjögren's Foundation has worked hard in providing us with evidence-based guidelines to answer these sorts of questions. I would go by the "Treatment Guidelines for Rheumatologic Manifestations of Sjögren's." The working group using the Delphi consensus process did all the hard work ...
How do you approach follow up of young patients with isolated +ANA, but no current clinical signs or symptoms of SLE?
An old study showed that ANA can be positive in patients who develop lupus up to 9 years (average 3 years) before the onset of clinical disease but it was not necessarily isolated ANA as Ro and La antibodies could also be detected long before the onset of the disease (Arbuckle et al., PMID 14561795)...
In patients with secondary Sjogren's how do you approach screening for lymphoproliferative malignancy?
1. Firstly, I do not discriminate between "secondary" and "primary" Sjogren's disease. There is currently a "Nomenclature Initiative" by the Sjogren's Foundation, led by Dr. Alan Baer, Director of the Sjögren’s Clinic at Johns Hopkins, and Dr. Manuel Ramos-Casals, a Sjogren's expert in Spain. Thus f...
Is there a role for immunosuppression in addition to standard PAH therapy in the management of pulmonary hypertension associated with primary Sjogrens syndrome?
In short: maybe. Pulmonary hypertension is quite uncommon in Sjogren’s. When I find it, I look for other causes of pulmonary hypertension, especially ILD, and for systemic sclerosis. I also consider other potential indications for immunosuppressive therapy such as inflammatory joint pain. There is, ...
How do you approach monitoring for lymphoproliferative disease in Sjogren's patients?
I have an increased index of suspicion for lymphoma, myeloma, and amyloid in Sjogren's. Increased age, longer disease duration, and greater disease activity increase the lymphoproliferative risk.Persistent unilateral parotid or other salivary gland enlargements, lymphadenopathy, vasculitis (purpura,...