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3 Myths, Pearls, and Tips Regarding Sjögren’s Syndrome

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Sherring. This is because the stretching of the lid may disrupt the delicate neural interconnections within the network of glands (these are the same glands that you stimulate when you massage your eyes). Another problem that we have encountered after blepharoplasty is increased zones of exposure keratitis. Particularly when sleeping, the lower lid may not make adequate contact with the upper lid, leading to a zone of increased evaporative loss and resulting desiccative injury.

Finally, a standard model for induction of keratoconjunctivitis sicca is Botox R [113].

3.5.6 Pearl

SS patients have unique and particular needs at the time of anesthesia and surgery.

Comments: Operating rooms typically have low humidity and patients are at risk for flare of their KCS or corneal abrasions. We have found this to be particularly true in the post-operative recovery room, where the patient is partially awake with ßuttering eyelids, receiving direct ßow of oxygen (usually not humidiÞed) to the face, and not really aware of their eye symptoms as they awaken from anesthesia. Therefore, we recommend the use of ocular lubricants during surgery to prevent complications.

Also, the anesthesiologist must be careful with the amount of anticholinergic agents given during intubation, as the SS patient may be unduly sensitive and develop inspissated secretions that cannot easily be cleared in the postoperative period (i.e., after abdominal or chest surgery) when raising tenacious secretions is difÞcult.

Finally, the use of oral saliva substitutes should be encouraged. It is expected that patients will be ÒNPOÓ (nothing by mouth) prior to many surgeries. In the absence of normal saliva, they will have unnecessary discomfort if they are not allowed to have their artiÞcial saliva. We have found this particularly true when the patient is a Òlate in the dayÓ case for elective procedures such as joint replacement.

3.5.7 Pearl

A wide range of peripheral neuropathies may be present in SS and constitute one of the most difficult aspects to treat.

Comments: Early attention to peripheral neuropathies is extremely important. The types of neuropathy include sensory including pure sensory and ganglionic neuronopathy. Sural nerve biopsy [114] frequently shows vascular or perivascular inßammation of small epineurial vessels (both arterioles and venules) and in some cases necrotizing vasculitis. Loss of myelinated nerve Þbers was relatively common and loss of small diameter type I nerve Þbers occurs.

Pathology in cases of sensory (doral root ganglion) consists of loss of neuronal cell bodies and inÞltration of T cells [114]. Also, peripheral motor neuropathies can include mononeuritis multiplex (that derives from vasculitis) and CIPD (Òchronic idiopathic peripheral demyelinationÓ) associated with anti-MAG (myelin-associated glycoprotein) disease. In addition, patients may suffer from trigeminal and other cranial neuropathies, autonomic neuropathy, and mixed patterns of neuropathy [114].

Sural nerve biopsy may show vascular or perivascular inßammation of small epineurial vessels (both arterioles and venules) and in some cases necrotizing vasculitis [114]. Loss of myelinated nerve Þbers is common and loss of small diameter nerve Þbers occurs. Peripheral neuropathy in PSS often is refractory to treatment although newer biological agents may provide more effective treatment options.

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