Sjogren's Syndrome: What does the hair specialist need to know?

Sjogren’s Syndrome: What does the hair specialist need to understand about this condition? 

Sjogren’s syndrome is an autoimmune disease that leads to a wide variety of symptoms, including dysfunction of the exocrine glands. This leads to two common symptoms of the condition - dry mouth and dry eyes. Dry mouth is referred to as “xerostomia” and dry eyes is referred to as “xerophthalmia.” Arthritis is also frequently present in patients with Sjogren’s leading to some to refer to the classic triad of Sjogren’s as dry mouth, dry eyes, and arthritis. Other organ systems of the body may be involved. Early recognition of this condition is extremely important to help prevent complications such as corneal ulceration, dental cavities, mouth infections and damage to various organ systems of the body. Early recognition will also allow the patient to receive proper monitoring for lymphoma which is known to be increased in patients with this disease. See article Sjogren’s Syndrome: Easy to Screen and Just as Easy to Forget

Epidemiology of Sjogren’s Syndrome

Sjogren’s syndrome may affect 0.5 to 2 % of the adults population. About 1/2 of people who have SS are undiagnosed. These statistics translate into SS being a fairly common autoimmune issues. Women are affected about 9 times more frequently than men.  The mean age of onset in one study was 52.7 years.

With primary Sjogren’s disease, the patient has no other autoimmune diseases present. With secondary Sjogren’s syndrome, the patient has other autoimmune issues or immune issues present. Rheumatoid arthritis for example, is commonly associated with secondary Sjogren’s. Systemic lupus, systemic sclerosis, mixed connective tissue disease and HIV are other examples of conditions that may be associated with secondary Sjogren’s syndrome.  In fact, about 25 % of patients with rheumatoid arthritis and systemic lupus have features of Sjogren’s syndrome.

Cause of Sjogren’s Syndrome

The cause of Sjogren’s is still not entirely clear. it is viewed as an autoimmune disease and both B cells and T cells have a role in this disease. Auto antibodies are found to various organs including salivary glands, thyroid glands, stomach mucosa, red blood cells, pancreas, prostate and nerve cells. It is thought that there is some type of immune dysregulation that also leads to neural type signals not reaching target organs in the correct manner. An important issue that occurs in Sjogren’s syndrome is dysfunction of the exocrine glands leading to dry mouth and dry eyes. The cause of the neuropathy is not entirely clear and may differ depending on the exact neurological issues the patient has

Signs and Symptoms of Sjogren’s Syndrome

Patients with Sjogren’s syndrome present with a wide array of symptoms. The symptoms are quite non specific sometimes and vague in the early stages. This leads to a delay in diagnosis. It’s not uncommon for symptoms to be first ascribed to menopause or other non specific phenomena such as medication-induced dry mouth or anxiety. In fact, some patients may have a delay of up to 10 years before finalizing receiving the correct diagnosis. Some present with issues relating to ‘dryness’ but some present to the doctor with neurological issues or internal organ issues. 

The most well-known are the dry mouth and dry eyes, so we’ll examine these first.

Dry mouth is present in a very large proportion of patients with SS. In some studies it may be as high as 93.5-98 %. However, one need to keep in mind that not everyone has dry mouth. One study suggest that 15 % the those with primary SS and 26 % of those with secondary SS did not have dry mouth. Dry mouth causes difficulty swallowing food without water. It may even bee difficulty for patients to speak. The patient may have an increase in dental caries (“cavities”) due to the dry mouth and lack of bacteria fighting saliva. Lack of saliva not only predisposes to bacteria but fungi as well. Oral candidiasis may also be present. A fissured tongue is frequently seen. Salivary glands may be swollen an often starts on one side before becoming bilateral.

Dry eyes are also common and affects up to 67 -93 % of patients in some studies. Patients usually need eye drops. Patients describe a variety of eye symptoms including itching, grittiness, soreness, dryness. Often the eyes have a normal appearance. Patients may have photosensitivity, redness, eye fatigue, and may report a sensation that they have a film across the eyes. Blepharitis may be present and may need antibiotics to address the Staphylococcus that is frequently present.  Low humidity and smoking will exacerbate symptoms. Consultations with ophthalmology are needed. Corneal ulcerations, vascularization and even perforation can occur.

In addition to dry eyes and dry mouth, patients with Sjogren’s may have a wide array of other symptoms.

Other systems can bee affected by the dryness. Dry skin is an example. Vaginal dryness is another example and may lead to painful intercourse (known as dyspareunia). Dryness may occur in the nose, throat and trachea. The phenomenon of a dry trachea is known as “xerotrachea” and may lead to a chronic dry cough. Other causes of cough are also possible other than reasons due to dryness (i.e. interstitial lung disease).

Arthritis can occur in primary Sjogren’s syndrome (or overlap with rheumatoid arthritis). Fatigue, brain fog and neuropathy are other symptoms that require evaluation. A variety of dermatological manifestations may occur including palpable purpura.

The Neuropathy of Sjogren’s Syndrome

The neuropathy of Sjogren’s is extremely complex. There are a wide range of neurological manifestation of Sjogren’s syndrome ranging from pins and needles type symptoms to neurological issues that closely resemble the findings in multiple sclerosis. In general the neurological issues include: 1) sensory ataxic neuropathy 2) multiple mononeuropathy 3) painful sensory neuropathy 4) trigeminal neuropathy (giving facial palsy) 5) autonomic neuropathy and 6) radiculopathy. 

The most common neurological issues seen in Sjogren’s is numbness and burning in the extremities that is caused by a sensory neuropathy or so called small fiber neuropathy. The neurological symptoms usually happen in the week to even years after the dry mouth and dry eyes occur but can sometimes even happen before the main symptoms of primary Sjogren’s syndrome gets diagnosed. 

Diagnostic Criteria for Sjogren’s Syndrome

There are many diagnostic criteria that have been proposed.  In fact, in the past 4 decades at least 11 criteria have surfaced.

A] AECC for Sjogren’s Syndrome

If one follows the American-European Consensus Criteria for Sjogren’s Syndrome, the diagnosis of Sjogren’s requires 4 of the following 6 symptoms (and one must be either a positive salivary gland biopsy result or antibodies to SSA or SSB). Any 3 of the criteria 2, 4,5,6 may also be used. 

1. Symptoms of dry eyes 

a) dry eyes for at least three months

b) a foreign body types sensation in the eyes

c) used of artificial tears more than 3 times per day

2. Signs of dry eyes 

a) abnormal Schirmer test

b) positive vital dye staining of the eye surface (Rose Bengal tests)

3. Symptoms of dry mouth

a) dry mouth for at least three months

b) recurrent or persistently swollen salivary glands

c) need for liquids to swallow dry foods

4. Positive salivary gland biopsy (labial biopsy) meeting criteria for Sjogren’s 

a) lymphocytic sialoadenitis with focal score >1 per 4 mm2)

5. Salivary gland functional test

a) unstimulated whole salivary flow less than 1.5 mL in 15 minutes

b) abnormal parotid sialography

c) abnormal salivary scintigraphy

6. Positive SSA (anti-Ro) or SSB (anti-La) antibodies or both

About 70 % have SSA antibodies and 50 % of those with SSA antibodies have SSB antibodies. Isolated SSB antibodies are rare. Some criteria also take into around positive RF and ANA but these are not part of the American-European consensus criteria. 

B] American College of Rheumatology (ACR) Criteria for Sjogren’s Syndrome

The ACR criteria represent a second diagnostic critieria. A benefit of the newer ACR Criteria over the AECC criteria discussed above is that it does not rely on patient symptoms in making the diagnosis. The criteria are strictly objective. If one follows the ACR criteria, there is a requirement to identify at least 2 out of the following 3 criteria:

1. Positive serum anti-SSA and/or anti-SSB or [positive rheumatoid factor and ANA ≥ 1:320].

2. Ocular staining score ≥ 3.

3. Presence of focal lymphocytic sialadenitis with focus score ≥ 1 focus/4mm2 in labial salivary gland biopsies.

Sjogren’s Syndrome: Issues that the Hair Specialist May Encounter.

A) Hair Loss from Telogen Effluvium

A telogen effluvium or “hair shedding” issue may occur with any systemic autoimmune issue including Sjogren’s syndrome. Generally speaking, the more dramatically that the immune system is activated or the great number of organs that are involved, the greater the risk of hair shedding. Patients with secondary SS who have co-existent rheumatoid arthritis or lupus or systemic sclerosis or mixed connective tissue disease may experience shedding from those diseases. The treatment involves treating the underlying autoimmune disease. If thyroid issues, low iron, abnormal blood levels, or stress aree contributing to thee shedding these issues need to be addressed. If a medication is causing the shedding (for example methotrexate in a patient with lupus), this needs to be addressed.

B) Hair Loss from Scarring Alopecia

Patients with secondary Sjogren’s have a variety of autoimmune issues. The exact frequency of scarring alopecia in patients with secondary Sjogren’s is not known but one must always be on the lookout for coexistent discoid lupus, frontal fibrosing alopecia (FFA) and lichen planopilaris (LPP).

C) Sicca Syndrome

The hair specialist needs to understand the meaning of Sicca syndrome as it is often confused with Sjogren’s syndrome. It is possible for a patient to have dry mouth and dry eyes without having an underlying autoimmune disease like Sjogren’s syndrome. In these cases, we refer to the patient’s dry mouth and dry eyes as ‘sicca syndrome” or “sicca complex” Many patients with scarring alopecias such as FFA and LPP have dry eyes as a symptom - and a smaller proportion have dry mouth as well. In many cases, the totality of their symptoms and signs does not fit perfectly with what we call Sjogren’s syndrome.

Many of these patients with dry eye many even have meibomian gland dysfunction and have many symptoms shared with patients with Sjogren’s syndrome.

When dry eyes and dry mouth occur in the absence of clear underlying autoimmune disease of primary or secondary Sjogren’s we use the term “sicca syndrome” or “sicca complex.”

D) Scalp Burning of Sjogren’s Syndrome 

Many patients with Sjogren’s syndrome report burning sensation in the scalp. The symptom of scalp burning in Sjogren’s is very poorly understood. Some patients question whether the burning is due to products and shampoos they use and some question whether it’s due to medications they use or general shedding problems they might be experiencing.

While it’s true that these can be the cause of scalp burning in a patient with Sjogren’s syndrome, the burning in some patients is frequently related to a neuropathy. We have a bit of work to do to understand the epidemiology of this burning symptom and how best to treat it. At present, we don’t really know the ideal treatment for the scalp burning of Sjogren’s syndrome. Gabapentin, amitriptyline, low dose naltrexone can be considered to help the burning scalp. In some cases, especially those situations where the neuropathy is linked to vasculitis, immunosuppressants may be needed. In these cases, hydroxychloroquine, methotrexate and cyclosporine can be considered.  One needs to exercise caution when prescribing immunosuppressants given the risk of lymphoma in patients with Sjogren’s syndrome. Low vitamin D has been shown in some studies to worsen the neuropathy and must be addressed. In addition, poor sleep has a significant impact in some patients on various aspects of their neuropathy. Efforts to help improve sleep quality are often beneficial. 

Conclusion

Sjogren’s syndrome is often viewed as a disease complex having the classic trial of dry mouth, dry eyes and arthritis. I hope I have illustrated that this simplistic view does not capture the complex autoimmune that Sjogren’s syndrome truly is. Patients have a range of systemic issues including those that affect the lungs, brain, joints, kidneys, and small nerves. Consultation with rheumatologists, dentists, and ophthalmologists is very helpful for most patents with Sjogren’s syndrome but other specialtiees may become involved in th care of the patient.

References 

Perzynska-Mazan et al. Neurological manifestations of primary Sjögren’s syndrome. Reumatologia. 2018; 56(2): 99–105. (Please see link for reference). 

SC Shiboski et al. American College of Rheumatology Classification Criteria for Sjögren’s Syndrome: A Data-Driven, Expert Consensus Approach in the SICCA Cohort. Arthritis Care Res (Hoboken). 2012 Apr; 64(4): 475–487. (Please see link for reference)