Sjögren’s disease

This disorder of the exocrine glands explains the appearance of dry syndrome characterised by dry eyes and mouth. 

Sjögren’s disease is characterised by the dysfunctioning of the exocrine glands (one speaks of “exocrinopathy”), which is the cause of the dry eyes and mouth but also dryness of the nose, throat, bronchi, skin and vaginal mucosa. This generalised dryness is very debilitating, having a negative impact on quality of life and can be the source of local complications: dry keratoconjunctivitis, oral mycosa, infection of the salivary glands, etc.

Sjögren’s disease is also a systemic autoimmune disease that can affect virtually all the organs. These so-called extraglandular manifestations can result from three different mechanisms: 1/ The appearance of a lymphocytic infiltration in other tissues than the exocrine glands (primary biliary cirrhosis, renal tubular impairment, bronchial damage) 2/ autoimmune  impairment linked to the presence of immune complexes or specific autoantibodies and 3/ an uncontrolled proliferation of chronically stimulated B lymphocytes (lymphocytic interstitial pneumonia, lymphoma).

Epidemiology

After rheumatoid arthritis,  Sjögren’s disease is the most common connective tissue disease with a prevalence of    0.1-0.6% in the general population. It is a disease that affects primarily women (9 women for 1 man), generally aged between 40 and 50. Its appearance around the time of the menopause in women and its relatively unspecific manifestations (dryness, pain, tiredness) explain why it is often late in being diagnosed. 

The trio of dryness - tiredness - pain (Sicca Asthenia Polyalgia)

The trio of dryness – tiredness – pain, known by some as SAP (Sicca-Asthenia-Polyalgia) syndrome, are the main complaints expressed by patients with Sjögren’s disease. As the disease appears in the exocrine glands, it is not surprising that almost 96% of patients present eye and mouth dryness that is often very debilitating. This dryness can be more general and affect the respiratory tracts (cough) and vaginal mucosa (intimate dryness in women). More than 50% of  Sjögren’s disease patients report tiredness as a symptom. Finally, almost 1 in 2 patients have chronic joint and muscular pain without any clear anatomical cause, making it similar to fibromyalgia syndrome. These “SAP” manifestations can be very debilitating, preventing the patient from working and considerably reducing the quality of life of patients with Sjögren’s syndrome.

Extraglandular manifestations of autoimmune origin

Although often reduced to its symptomatic trio as set out above,  Sjögren’s disease nevertheless remains a systemic rheumatological disease of autoimmune origin that can affect any organ of the body, sometimes very seriously. In regard to this dimension, it is close to its cousin:

disseminated lupus erythematosus. Sjögren’s disease can thus manifest through a Raynaud phenomenon, constitutional manifestations (fever of unexplained origin, weight loss), glandular manifestations (major swelling of the  protid and submaxillary saliva glands), ganglionary manifestations (swollen glands known as adenopathies), joint manifestations (inflammatory pain with morning stiffness for more than 30 minutes, polyarthritis),   muscular manifestations (inflammatory myositis), neurological problems (polyneuropathies, brain vasculitis, symptoms miming  multiple sclerosis), bronchial lung damage or damage to lung tissue, kidney damage (proteinuria, renal failure, glomerular lesions, tubulointerstitial lesions complicated by tubular acidosis or osteomalacia), autoimmune haematological manifestations (anaemia, thrombocytopenia, leukopenia) and biological haematological manifestations (cryoglobulinemia, complement consumption, hypergammaglobulina).

Non-Hodgkin’s lymphoma

The intense activation of the immune system can lead to a major increase in B lymphocytes. If these lose their regulating mechanism, a B cell sub-population will continue to proliferate without limit: this is a cancer of the immune system, known as non-Hodgkin’s lymphoma. It is a complication found in between 5 and 10% of Sjögren’s disease patients. Generally slow to develop and quite discreet, it should be sought in the case of a chronic unilateral swelling of a salivary gland, adenopathies or an unexplained fever, for example. The rheumatologist may screen persons at risk of developing a lymphoma subsequent to  Sjögren’s disease by searching for clinical and biological anomalies reflecting the appearance of an excessive B-cell proliferation and monitor them more closely. 

Due to its frequent non-specific manifestations  - tiredness, pain and dryness – and its systemic manifestations that can be polymorphous (neurological, kidney, lung, skin disorders, etc.) Sjögren’s disease can be complicated to diagnose.  In addition, there is not at present any single test that permits a diagnosis.

The diagnosis is based on a set of clinical and biological arguments: 

• Subjective dryness of the eyes and mouth;
• Objectification of an impaired functioning of the salivary and lachrimal glands  

- Establishing of damage to the surface of the eye by an ophthalmologist ;

- Establishing of a reduced lachrimal flow by the Schirmer test;

- Establishing of a reduced salivary flow by a sialometry;

- Establishing of a malfunctioning of the salivary glands by a scintigraphy;

- Establishing of MRI or ultrasound anomalies in the salivary glands.

• Lymphocytic Infiltration on a biopsy of accessory salivary glands;
• Establishment of anti-Ro/SSA and/or anti-La/SSB autoantibodies in the blood.

Treatment

There is not at present any curative treatment for Sjögren’s disease.

Treatment is based on 3 dimensions:

The dryness syndrome, tiredness and chronic pain (evaluated by the ESSPRI score) are treated on a  symptomatic and multidisciplinary basis in cooperation with the Departments of Ophthalmology and Physiotherapy and the Pain Clinic. The initial evaluation aims to exclude the involvement of certain manifestations systemic to tiredness and pain  (hypothyroidism, osteomalacia, lymphoma) as well as to identify persistent limiting factors (sleeping problems, depression, kinesiophobia, etc.) before proposing a multimodal treatment.  These interventions include prescribing artificial tears or saliva and the use of eye drops based on an autologous serum.  Pilocarpin is also a medication commonly prescribed to permit an increase in tear or saliva secretions. Sjögren’s disease is recognised as a “serious pathology” that brings the entitlement to 60 physiotherapy sessions a year. Following a diagnosis by your rheumatologist your mutual insurance company can also grant you monthly financial assistance to purchase eye drops and other medicines against dryness.

Extraglandular manifestations of an autoimmune origin (evaluated with the aid of the ESSDAI score) can be present to very variable degrees. Depending on the organs affected and the seriousness of the inflammation, an immune suppression / immune modulator treatment may be prescribed by the rheumatologist: corticoids (Médrol), hydroxychloroquine (Plaquenil), méthotrexate, azathioprine, mycophenolate mofetil, rituximab or cyclophosphamide. Within our institution, the multidisciplinary treatment of complex systemic diseases   - of which Sjögren’s disease is one – is one of the missions  of the  MISIM (Immuno-Mediated Systemic Inflammatory Diseases) platform that includes rheumatologists, general internists, nephrologists, pneumologists, dermatologists... with a penchant for and expertise in autoimmune diseases.

Sjögren’s  disease – apart from the intense activation of B lymphocytes – can be complicated in certain patients by a non-Hodgkin’s lymphoma (cancerous proliferation of the lymphocytes). This risk is the reason for at least an annual clinical and biological follow up in patients with Sjögren’s disease. If a lymphoma appears  in a patient being monitored for Sjögren’s disease, the patient will be monitored in cooperation with our hospital’s Department of Haematology.