Aravind Palraj

🧵 Neuro-Sjögren’s: Beyond Dryness

Sjögren’s Syndrome isn’t just sicca.

When nerves get involved, it can be devastating — and often misdiagnosed.

Here’s a deep dive into neurological manifestations of Sjögren’s Syndrome👇

#Rheumatology #NeuroSjögren #MedTwitter

1️⃣ How common is neuro involvement in Sjögren?

📊 ~20–30% of patients with primary Sjögren’s may develop neurological symptoms.

It can precede dryness. It can mimic other diseases.
That’s why it’s called a “great imitator.”

Peripheral &gt; Central nervous system involvement.

2️⃣ Peripheral Nervous System (PNS) Manifestations

🧠 The most common neurological feature.

🧩 Types:
• Sensory ataxic neuropathy
• Small fiber neuropathy
• Mononeuritis multiplex
• Cranial neuropathies (esp. CN V, VII)
• Autonomic neuropathy
• Trigeminal sensory

3️⃣ Small Fiber Neuropathy (SFN)

🔍 Often missed.

Symptoms:
• Burning pain
• Dysesthesia
• Allodynia
• Normal NCS/EMG

📌 Confirmed with skin biopsy (↓ intraepidermal nerve fiber density) or QST.

🧠 Strongly associated with fatigue and autonomic dysfunction.

4️⃣ Sensory Ataxic Neuropathy

🎯 Most specific for Sjögren.

• Loss of proprioception
• Positive Romberg sign
• Falls, imbalance
• Normal motor strength

MRI may show dorsal root ganglia enhancement.

🧪 May be the presenting symptom of primary Sjögren.

5️⃣ Trigeminal Neuropathy (CN V)

📍 Presents as facial numbness or paresthesia.

Bilateral or unilateral.

⚠️ Often misdiagnosed as MS, especially if MRI shows nonspecific white matter lesions.

Red flag = demyelinating mimicry in Sjögren.

6️⃣ Autonomic Neuropathy

🌀 Affects:
• BP regulation → orthostatic hypotension
• GI motility
• Bladder function
• Heart rate variability

Often overlaps with POTS or SFN.

🩺 Requires autonomic testing (HRV, QSART).

7️⃣ Mononeuritis Multiplex

⛔ Acute, painful, asymmetric neuropathy.

Often due to vasculitis of vasa nervorum.

Check:
• ESR, CRP
• Cryoglobulins
• Complement levels
• Nerve biopsy if needed

Treat aggressively — this is organ-threatening.

8️⃣ CNS Involvement in Sjögren

Less common, but serious.

🧠 Can resemble:
• MS
• NMO spectrum disorders (anti-AQP4)
• ADEM
• Cerebral vasculitis
• Transverse myelitis
• Cognitive dysfunction (“brain fog”)

⚠️ Demyelinating lesions + sicca = screen for Sjögren!

9️⃣ Anti-Ro antibodies and CNS Sjögren

💡 Anti-Ro/SSA often positive, but not always.

Anti-AQP4 and MOG should be tested in myelitis or optic neuritis.

CSF:
• Mild lymphocytosis
• ↑ protein
• Oligoclonal bands (can mimic MS)

🔟 Neuropsychiatric &amp; Cognitive Involvement

🧠 “Brain fog” is real.

Can manifest as:
• Memory issues
• Difficulty concentrating
• Depression
• Anxiety

PET scans may show hypometabolism in frontal/temporal lobes.

🧠 Mechanism: chronic inflammation + cytokines.

1️⃣1️⃣ Diagnosis of Neuro-Sjögren

🧪 Workup:
• ANA, anti-Ro/SSA, anti-La/SSB
• Cryoglobulins, complements
• Lip biopsy (focus score ≥1)
• Schirmer’s, salivary USG
• NCS/EMG, skin biopsy, MRI brain/spine

🧠 Often needs multidisciplinary input — neurology + rheumatology.

1️⃣2️⃣ Treatment Principles

Depends on phenotype:

✔️ SFN / mild sensory – Gabapentinoids, duloxetine, IVIG (in select cases)

✔️ Mononeuritis multiplex / vasculitic neuropathy – Steroids + cyclophosphamide or rituximab

✔️ CNS involvement – High-dose steroids, immunosuppressants

1️⃣3️⃣ Prognosis

✴️ Early recognition = better outcomes.

✴️ Delay can lead to permanent damage — esp. in dorsal root ganglionopathy.

✴️ Autonomic and small fiber neuropathy may persist despite treatment.

Don’t wait for dryness. Think Sjögren early.

📍Key Clinical Pearls

• Neuro symptoms may precede dryness.
• SFN = common + often overlooked
• Trigeminal neuropathy is a red flag.
• CNS Sjögren can mimic MS/NMO
• Skin biopsy can be diagnostic when NCS is normal
• Always treat vasculitic forms aggressively.

🙏 Thanks for reading!

Neuro-Sjögren is underdiagnosed, often misunderstood, and yet profoundly disabling.

If you see a patient with unexplained neuropathy, don’t forget to screen for Sjögren’s — even if dryness isn’t the headline.

#NeuroSjögren #RheumTwitter #SjögrensSyndrome

Skin biopsy ≠ rule-out SFN
SFN can be clinically diagnosed 
Will work on a thread for neuro-APS soon.