Abstract
The main orofacial manifestation of the inflammatory rheumatic diseases is that of Sjögren’s syndrome. In addition, there is a constellation of orofacial manifestations of the inflammatory rheumatic diseases, many of which are extra-articular with some constituting presenting signs of the underlying rheumatic disease. This review will discuss the orofacial manifestations in a variety of connective tissue diseases and will also allude to the oral adverse drug reactions that may occur as a consequence of therapy.
Introduction
This review will provide an overview of the orofacial manifestations of the inflammatory rheumatic diseases and will briefly allude to the oral adverse reactions of drug therapy used in the management of these conditions.
Sjögren’s Syndrome is the most widely recognised orofacial manifestation of the connective tissue diseases, although a variety of other clinical features affecting the orofacial region may also occur in these diseases. In some cases, they may represent an initial presenting sign. In addition to the conditions themselves, iatrogenic pathology as a consequence of both traditional, as well as the newer biological therapies, may manifest in the orofacial region.
Rheumatoid arthritis frequently involves the temporomandibular joints, although such involvement is often asymptomatic. Systemic lupus erythematosus may produce the well-described malar flare or so called ‘butterfly rash’ as well as oral ulceration. Systemic sclerosis produces orofacial manifestations in approximately 80% of patients, and in one-third of these patients, orofacial involvement may be the presenting sign. Whilst Raynaud’s phenomenon typically affects the extremities, tongue involvement has also been reported. Neurological manifestations of the connective tissue diseases in the orofacial region include isolated sensory trigeminal neuropathies and trigeminal neuralgia-like pain. Oral ulceration is typically seen in Behçet’s disease and reactive arthritis, whilst orofacial manifestations may occur in the vasculitides, including characteristic gingival involvement in granulomatosis with polyangiitis (Wegener’s granulomatosis) and lingual infarction in temporal arteritis.
Sjögren’s syndrome
Sjögren’s syndrome, an autoimmune exocrinopathy, was first described by Henrik Sjögren, a Swedish Ophthalmologist, in 1933 . The major clinical features are those of dryness of the eyes, mouth and other mucosal surfaces, together with various other manifestations, including arthralgias and frequently profound, chronic fatigue . This is known as Primary Sjögren’s syndrome, whilst ‘Secondary Sjögren’s syndrome’ is used when these features occur in association with another inflammatory rheumatoid disease or autoimmune condition such as primary biliary cirrhosis or autoimmune thyroid disease.
The diagnosis of Sjögren’s syndrome is essentially clinical, based on both clinical presentation and laboratory investigations. Various classification criteria have been developed and although primarily intended for research cohorts, they provide useful guidance for diagnosis. The revised American European Consensus Criteria, Vitali et al., 2002 , are widely used, but more recently, Shiboski et al., 2012 have published further classification criteria, which are endorsed by the American College of Rheumatology. These latter criteria are dependent on objective criteria such as minor salivary gland biopsy, serology and objective ophthalmological assessment. They do not consider symptomatology.
Orofacial features of Sjögren’s syndrome
Oral symptomatology
Xerostomia is the major oral symptom of Sjögren’s syndrome, although when Sjögren’s syndrome occurs in individuals below 40 years of age, dryness is often not a significant symptom because of the degree of salivary reserve (Scott ). These patients are more likely to complain of painless swelling of the parotid (particularly lower pole) and/or submandibular salivary glands.
Other symptoms due to reduced salivary flow include reduced taste perception, difficulty in swallowing and speech, poor denture retention, oral malodour, increased rate of dental decay, and possibly more marked periodontal disease, generalised oral discomfort and salivary gland swelling that may or may not be painful dependent on its cause. Various neurological features may be seen in some patients, with isolated trigeminal sensory neuropathies being reported in approximately 5% of patients with primary Sjögren’s syndrome.
Such symptomatology has a significant effect on quality of life (Fox PC et al. Kamel et al., 2009 ).
Clinical signs of xerostomia
Extra-orally, xerostomia ( Fig. 1 ) may produce angular cheilitis, dry flaky lips and salivary gland swelling, often bilateral, involving any or all the major salivary glands. These signs are non-specific and other possible diagnoses must be excluded.
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