Thread by @SarahSchaferMD

#Sjogren’s is not rare. It is about as common as RA, but mostly undiagnosed. Prevalence estimates vary widely, from .1% to 3 %. Low estimates are derived from registries that do not account for underdiagnosis, under-reporting, and Sjogren’s w/ another AIRD. 1/18

About 1% of the US population has #Sjogren’s. It is a neglected disease, despite undeniable evidence that it is a serious, systemic disease. The “rare, nuisance sicca disease” paradigm remains deeply entrenched, even among some rheumatologists. 2/

https://twitter.com/SarahSchaferMD/status/1550897555126382592

Primary care, eye, and dental providers often encounter early signs of the disease but do not recognize them because Sjogren’s medical education is largely absent. It takes 10 years, on average, before Sjogren’s is diagnosed following a dry eye disease diagnosis. 3/

PCPs who see typical systemic symptoms rarely screen for sicca. I have taught > 200 PCPs about #Sjogren’s. I ask them questions before my presentations. Not one PCP had ever suspected Sjogren’s. Fewer than 5 thought they had ever encountered a Sjogren’s patient. 4/

This is a system problem, not a lack of interest. With almost no #Sjogren’s education, clinicians logically assume it is rare and unimportant. Lack of familiarity w/ Sjogren’s deprives patients of diagnosis and proper care. Thanks to @DrChadJohr @lupusenclopedia for teaching 5/

Where are all the missing Sjogren’s patients? Items 1- 9 illustrate many of the reasons that Sjogren’s is largely undiagnosed. For details, including citations, see this page of Sjogren’s Advocate: 6/
sjogrensadvocate.com/sjogrens-is-co…

1⃣ Typical presentations are often missed. Some combo of dryness, fatigue, neuropathy, GI, or joint pain occurs in most. #Sjogren's is often misdiagnosed as sicca syndrome, ME/CFS, fibromyalgia, IBS, FND, or anxiety. Patients usually do not mention sicca symptoms to PCPs. 7/

2⃣ Financial and practical barriers. #Sjogren’s is a complex disease. Even people w classic symptoms usually see multiple clinicians for years before being diagnosed. This is time-consuming, disheartening, and may not be financially feasible, especially in the U.S. 8/

3⃣ Younger patients. About half w/ #Sjogren’s can remember clear symptoms starting in childhood or early adulthood. Children and young adults often do not fulfill Sjogren’s classification criteria. By the time of most are diagnosed, the disease has progressed for years. 9/

4⃣ Men. 10% of #Sjogren’s patients are male. I know male patients who were told they can’t have Sjogren’s because it is a “women’s disease”. Sjogren’s is often called a “disease of middle-aged women”. This is wrong b/c males do get it & it often starts younger. 10/

5⃣ Early systemic involvement. These patients are often misdiagnosed w/ SLE, RA. Patients with inflammatory arthritis from Sjogren’s are often assumed to have RA. RF is positive in at least half of Sjogren’s patients who do not have RA. 11/
ncbi.nlm.nih.gov/pmc/articles/P…

6⃣ Non-sicca presentations (20%). Usually undiagnosed for years until sicca appears unless a very astute clinician picks it up. People I know in this group: CNS demyelinating disease, aseptic meningitis, PNS including POTS, pericarditis, ILD, cytopenia, cutaneous vasculitis. 12/

7⃣ “Secondary” Sjogren’s, a misleading term that should be discarded. Up to 50% w #Sjogren’s have another AIRD. Sjogren’s is often overlooked when called “secondary” despite it being a distinct, well-characterized disease w a higher rate of lymphoma & other neuropathies. 13/

8⃣ SSA/SSB or ANA negative patients. 30-40% are SSA neg. Unfortunately, many rheumatologists refuse to diagnose #Sjogren’s w/o typical autoantibodies, even when patients fulfill classification criteria. Seronegative Sjogren’s is also serious. 14/
sjogrensadvocate.com/post/do-you-ha…

9⃣ Clinical picture of #Sjogren’s but not meeting classification criteria b/c SSA neg & lip bx neg or absent. “Negative” biopsies are often abnormal. Non-specific findings such as diffuse lymphocytic infiltration, fibrosis & atrophy are common in Sjogren’s but don’t count. 15/

My own story illustrates why #Sjogren’s is a big picture diagnosis, not just blood tests. I’m SSA/ANA neg. Symptoms started in teens, building through 20s and 30s➡️incapacitating systemic symptoms at age 44. First called ME/CFS but diagnosis confirmed at 52 w/2nd lip biopsy. 16/

My lip biopsy (MSGB) age 44: diffuse lymphocytic infiltration, FS 0. Repeat MSGB age 52: FS 1.5. 3rd MSGB age 54 for a study: fibrosis and scarring, FS 0. I was lucky to hit the right moment with the 2nd positive biopsy and fortunate my rheum had treated me “as if”. 17/

▶️My story is not unique.
▶️Treat patients, not checkboxes.
▶️Seronegative Sjogren’s patients can be extremely ill.
▶️Rheumatology practice and medical education need to reflect 21st-century knowledge.
▶️Sjogren’s is a common, serious disease, never limited to sicca. 18/18

@DrChadJohr
@lupuscyclopedia
@Lupusreference
@SjogrensCa
@SjogrenEurope
@SjogrensOrg
@SjogrensForum
@MdStens
@SjogrenpowerAna

* oops. that should be neuropathies. I know lymphoma is not a neuropathy!