Sjögren's syndrome is a slowly progressive autoimmune inflammatory disease that is associated with destruction of the exocrine glands (salivary glands) and can lead to the absence or reduction of secretions in many places in the body.

Sjögren's syndrome may, in some cases, be associated with systemic diseases involving the lungs, liver and kidneys.

Despite the fact that the incidence of pulmonary problems in the disease was described by Henrik Sjögren more than fifty years ago, it continues to be largely underdiagnosed even today. There are descriptions of patients with Sjögren's syndrome presenting with several pulmonary complications, including bronchial complications, bronchiolitis, interstitial pneumonia, and lymphoma.

The spectrum of pulmonary complications can be even wider if we consider that secondary Sjögren's syndrome can coexist with any other disease of the autoimmune system.

In the case of primary Sjögren's syndrome, the true frequency of pulmonary complications is not well defined.

Bronchitis and dry trachea (xerotrachea): The respiratory tract appears to be the most common target in patients with primary Sjögren's syndrome. In one study, obstruction was noted in 25% of cases of primary Sjögren's syndrome observed. The obstruction of the small ducts in these cases is probably the result of infiltration by inflammatory cells similar to those found in the salivary glands.

The most frequently observed clinical occurrence linked to a problem with the respiratory tract is that of a dry cough which is present in 40% of patients and which can be of variable intensity. This cough is usually linked to the dryness observed in the bronchial tree which is due to the inability of the salivary glands of the bronchial tree to secrete sufficient mucus.

This abnormality due to inflammatory bronchitis in Sjögren's syndrome patients is rarely significant when observed clinically.

Diagnosis can also be complicated because chest imaging methods, such as X-rays, CT scans or others, often reveal nothing despite the fact that the small airways are effectively blocked. These methods have only proven effective in the most advanced cases.

The discovery of lymphocytes in patients with active Sjögren's syndrome is not limited to the bronchial tree, but is also found in other organs such as the liver or kidneys. The term autoimmune epithelitis, which was given by Dr. H. Moutsopoulos to explain the abnormalities of the salivary glands as well as other glandular tissues (involved in Sjögren's syndrome), comes from these discoveries made in the lungs and other major organs.

Sjögren's syndrome lies at the intersection of immune system disease and lymphoid malignancy. For some patients, the disease covers a broad spectrum of symptoms ranging from benign tissue infiltration to malignant lymphoid infiltration. In some patients, benign lymphoid tissue is observed. This may present as “follicular bronchiolitis” which can mimic interstitial lung disease. This form usually responds very well to treatment with corticosteroids.

Lymphoid interstitial pneumonia is a lung problem that infiltrates the tissues. This is a classic but uncommon phenomenon of primary Sjögren's syndrome and may also be linked to other viral infections and other chronic abnormalities such as liver disease. To make the diagnosis, a long biopsy is necessary and, here again, this condition responds well to steroid therapy, accompanied or not by immunosuppressive drugs. Primary lymphoma in SS cases is uncommon and presents as a solitary or diffuse image on chest X-ray. To make this diagnosis, a lung biopsy is also necessary.

Interstitial pulmonary fibrosis

Pulmonary interstitial fibrosis is rather rare in patients with Sjögren's syndrome. In one study, 2 out of 343 patients were found to suffer from this abnormality. This is often associated with progressive scarification of the lungs. For this entity, we proceed with experimental medication.

Other less common entities, such as vasculitis and primary pulmonary hypertension, which involves the circulation in the lungs, are also very rare in Sjögren's syndrome. Other forms of bronchiolitis have also been described in Sjögren's syndrome, but they are also very rare.

Summary

Henrik Sjögren was the first to describe the involvement of the lungs in Sjögren's syndrome more than 50 years ago. Since that time, the incidence of pulmonary involvement in primary Sjögren's syndrome has been the subject of numerous studies, and several pulmonary complications have been described including bronchial diseases, interstitial pneumonia and lymphoma. Although common and presenting in several forms, complications of this type are rarely severe, when observed clinically, in patients with Sjögren's syndrome.

If the patient suffers from significant shortness of breath and changes are observed on the X-rays, a cellular diagnosis is imperative, i.e. a transthoracic lung biopsy. In light of these results, treatments are generally effective when initiated at the beginning of the disease.

Source: Moisture Seekers, May 2001
Thanks to Madame Lefrançois from the Volunteer Center for the translation of this text.

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Fatigue and Sjögren's syndrome

Fatigue is undoubtedly one of the most frequent and disabling symptoms in people with Sjögren's syndrome. This observation was well documented in a quality-of-life survey conducted five years ago by Dr. Evelyn Bromet, epidemiologist and member of the SSF Board of Directors. Questionnaires were sent out to all SSF members, and over 3,300 people responded. Members ranked fatigue as the third most troublesome symptom, after dry eyes and dry mouth. Skin dryness, musculoskeletal pain and poor sleep were also on the list. This article will review the main causes of fatigue I in Sjögren's syndrome, as well as the diagnostic approach and treatment options.

Definition

Fatigue is defined as a state of insufficient energy characterized by physical or mental weariness. It typically has fluctuating intensity in most patients since affected people can have good and bad periods during the same day. Some of the most important causes of fatigue in Sjögren's syndrome are listed here:

• Systemic inflammation
• Poor sleep
• Fibromyalgia
• Depression
• Drug side effects
• Hypothyroidism
• Myositis
• Corticosteroid-induced myopathy

Sleep disorders

The above-mentioned survey also indicated that the majority of Sjögren's syndrome patients are poor sleepers, and this can contribute to fatigue. Symptoms can include difficulty falling asleep, staying asleep or just waking up most of the time feeling tired, but not really aware of having a sleep problem (due to non-restorative sleep). In Sjögren's syndrome, sleep abnormalities may be a consequence of the disease itself, or related to the side effects of treatment. Causes of poor sleep include dry eyes, dry mouth, pain due to arthritis, poor sleeping habits or pain due to concomitant fibromyalgia. Frequent use of corticosteroids can also lead to insomnia. One study also suggested an increased incidence of restless legs syndrome in patients with Sjögren's syndrome compared with other groups. This syndrome is characterized by involuntary movements of the arms or legs during sleep, and can be difficult to recognize when sleeping alone. It can, however, be identified by performing a sleep analysis.

Treatment of sleep disturbances in Sjögren's syndrome must be tailored to the individual. Everyone should be encouraged to acquire good “sleep hygiene”, i.e. good sleep habits. For night-time eye discomfort, eye lubricants last longer than artificial tears and provide better relief. Regular use of secretagogues (a substance, whether biological or chemical, is said to be a secretagogue if it induces or increases the secretion of an endocrine gland) such as pilocarpine (Salagen) or cevimeline (Evoxac), one dose before bedtime, will reduce the need to ingest fluids during the day and the sleep disruption associated with the need to drink and urinate at night. Low doses of clonazepan (Klonopin, Valium) or carbidopa/levodopa (Sinemet) can effectively treat restless legs syndrome.

The best way to get a good night's sleep:

• Keep regular sleep schedules
• Exercise regularly, at least 20 minutes a day.
• Create a dark, cool, quiet sleeping environment.
• Take a warm bath to relax aching muscles or relieve stress at bedtime.

Not recommended:

• Take naps during the day.
• Take caffeine 6 hours before bedtime.
• Drink alcohol or smoke within 2 hours of bedtime.
• Exercise just before bedtime.
• Going to bed hungry or working in your room.

The second part of “Fatigue in Sjögren's Syndrome” will deal with depression, medication side-effects, hypothyroidism and muscle weakness, as well as presenting some new approaches to combating fatigue.

Depression

Depression can complicate all chronic illnesses, including Sjögren's syndrome, and may explain fatigue, pain, recent weight changes, headaches or other symptoms, when no other reason is apparent. In the course of their daily work, doctors can screen for the main symptoms of depression using mini-questionnaires. The patient is asked: “Have you felt any of the following almost every day for the past two weeks? "For example, sleep disturbance, anhedonia (loss of interest in doing things), low self-esteem or reduced appetite?

Studies have shown that the presence of two or more of the main symptoms is strongly correlated with a diagnosis of depression. Depression can be managed with psychological help, support group membership, regular physical activity and, in some cases, medication. (St John's Wort may help combat depression, but it can also aggravate dry mouth in patients with Sjögren's syndrome).

Hypothyroidism

Hypothyroidism (thyroid hormone deficiency) is common in people with Sjögren's syndrome. It is due to a combination of causes, including the autoimmune thyroid disease known as Hashimoto's thyroiditis.

What are the general symptoms of Hashimoto's disease?

• chronic fatigue
• weight gain,
• unusual thickening of the face and fingers,
• pale complexion, constipation,
• joint pain,
• nervousness,
• irritability.

How is Hashimoto's disease treated?

Hypothyroidism is treated by increasing thyroid gland production through the administration of a thyroid hormone called levothyroxine. This treatment also reduces the size of the enlarged thyroid gland.

Ways to combat fatigue

Irrespective of the cause, the usual means that help almost all patients combat fatigue include stress reduction, learning relaxation techniques, scheduling rest periods during the day, regular moderate physical activity (such as walking or swimming) and adapting daily life as best as possible.

Fatigue associated with Sjögren's syndrome is frequent and is due to multiple factors. Can a given patient have several causes of fatigue? An assessment is necessary to identify the cause(s) of fatigue. It may take several months to improve and learn to live with this frequent and disabling problem in Sjögren's syndrome.

Source: AFGS Bulletin

2024-06-10