Dr. Juan Gabriel Ovalles, a renowned specialist in Rheumatology, answers several key questions about Sjögren’s syndrome: what is it, what does it consist of, what are its symptoms, how is it treated?
What exactly is Sjögren’s syndrome?
Sjögren’s syndrome is a chronic systemic autoimmune rheumatic disease (SARS) that predominantly affects women, usually diagnosed between the ages of 40 and 60, but is also found in children, younger women and older adults.
According to the EPISER study of the Spanish Society of Rheumatology, the estimated prevalence of Sjögren’s syndrome in the adult population in Spain is 0.33%, approximately 125,000 cases nationwide. It is an immunological disorder in which our defense system attacks us producing inflammation and dysfunction of the exocrine glands that generate saliva, tears, respiratory and vaginal fluids, it can also generate recurrent enlargement of the parotid glands and up to 30% of patients develop extraglandular or systemic manifestations such as fatigue, musculoskeletal symptoms, skin lesions and involvement of internal organs and tissues (pulmonary, renal, hepatic, neurological and hematological). Not all cases are the same; the severity of the disease can vary over a wide range.
What symptoms indicate that a patient has this syndrome?
There is no single symptom that confirms the existence of the disease. The most frequent manifestation is the inflammation and dysfunction of the exocrine glands that produce tears, saliva and other mucosal fluids such as those of the genital tract and respiratory tract, giving rise to symptoms of oral, ocular, vaginal and respiratory tract dryness.
When these symptoms are prolonged over time, they can significantly deteriorate dental health, visual acuity, sexual relations and generate chronic irritative cough.
Some patients present with the disease without having previously developed dry eye or dry mouth. Other patients (up to 30%) present signs and symptoms in internal systems and organs such as hematological alterations, kidney or liver dysfunction, inflammation of the peripheral nerves (neuropathy) or central nervous system (myelitis, headaches, cognitive impairment, mood disorders).
What are the causes of this disease?
The exact origin is unknown, but the mechanisms of the disease are increasingly better understood. Sjögren’s syndrome requires a combination of environmental (smoking, viruses, ultraviolet light), genetic and hormonal factors to develop.
Recent data suggest the central role of the type I interferon system and B-lymphocyte growth and maturation factors (BAFF/BLyS) in the development of many autoimmune disorders, including Sjögren’s syndrome. A better understanding of the causes will lead to better treatments.
How is Sjögren’s syndrome detected?
The detection and accurate diagnosis of Sjögren’s syndrome is often a complicated task. The difficulty of detection is generated by the great variety of signs and symptoms (many of them non-specific) that lead to the patient’s pilgrimage through various specialists, also by the absence of a single diagnostic test (there is no blood test or test that confirms the existence of the disease), by the delay in access to consultation with the specialist in Rheumatology, because many cases are mild and go unnoticed or even by ignorance of the physician.
The diagnosis is made on the basis of a series of signs and symptoms justified by an inflammatory immune disorder that can be detected in blood tests (autoantibodies, inflammatory reactants, proteinogram alterations) and in salivary gland biopsy. Salivary gland ultrasound is a technique that seeks to minimize the use of invasive tests and improve early detection of the disease.
What is the most appropriate treatment?
As we have mentioned, Sjögren’s syndrome is not the same in all patients and the severity of the disease can fluctuate from mild cases that never consult a doctor to severe cases with systemic inflammation affecting internal organs such as lung and brain. This is why the most appropriate treatment must be individualized in each case, assessing the existing manifestations and their severity. As the severity of the disease increases, the intensity of the treatment should be increased.
In milder cases that only present dry eye discomfort, artificial tears are usually sufficient, then when the disease worsens and generates dry keratitis, the use of topical immunosuppressants (cyclosporine and glucocorticoids in eye drops) becomes necessary, for the manifestations of significant dryness, skin and joints, immunomodulators such as antimalarials (hydroxychloroquine) are used, which are sometimes associated with low doses of glucocorticoids.
For more resistant cases or those with systemic manifestations, immunosuppressive drugs are used to reduce the immunological disorder causing the organic damage.
In severe cases that do not respond to classic treatment, biological therapies, intravenous immunoglobulins and plasmapheresis are used. The ideal treatment is the one that starts early, avoiding delays, when the inflammation has already damaged the organs generating irreversible scars, it is too late and treatments are not effective. Early diagnosis is the key to effective treatment.
For more information, consult a Rheumatology expert.