Article Text

A case of secondary Sjögren’s syndrome with a swollen thigh
  1. Atul A Deodhar,
  2. Michael G Bruce,
  3. Kelly D Krohn,
  4. Robert M Bennett
  1. Division of Arthritis and Rheumatic Diseases (L329A), Oregon Health Sciences University, Portland, OR, USA
  1. Dr A A Deodhar, Division of Arthritis and Rheumatic Diseases (L329A), Oregon Health Sciences University, Portland, OR 97201, USA.

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Case report

A 73 year old English woman with seropositive, nodular, severe destructive rheumatoid arthritis (RA) and secondary Sjögren’s syndrome was seen with a 10 day history of pain and five day history of swelling in the left thigh.

She reported that she got out of bed 10 days ago, and felt pain in her left thigh, which she presumed to be a pulled muscle. She had difficulty in weight bearing and walking, but continued her already limited daily activities in a sheltered accommodation. Five days after this episode she noticed generalised swelling of her left thigh and developed fever (38.5°C), chills, headache, and malaise. The fever and associated symptoms resolved with high doses of acetaminophan, but the thigh pain and swelling progressively worsened to where she became bedridden. She had no history of swelling in her calves, recent surgery, prolonged immobilisation or any injury to her left thigh before the onset of pain.

Her past medical history was notable for debilitating RA of 30 years duration. Over the years, she had had bilateral hip arthroplasties and hand surgeries. She had developed a severe secondary Sjögren’s syndrome (diagnosis based on clinical criteria only) with corneal ulcerations despite regular use of artificial tears and protective glasses. She had lost all of her teeth except five; she wore dentures and used artificial saliva. Over the past eight years her RA was stable with prednisone 5 mg per day, methotrexate 7.5 mg orally per week, and nabumetone 500 mg twice a day. She was a widow and lived alone in a sheltered accommodation. She did not smoke. Her son and daughter in law who lived in the same town, helped her with shopping and transportation.

On examination, she was afebrile and had normal pulse and blood pressure. She had evidence of longstanding severe destructive arthritis in most of her joints of hands and feet including boutonnière and swan neck deformities in fingers and subluxation of all metatarsophalangeal joints with callus formations on soles. There was prominent nail bed vasculitis. She had bilateral chronic synovitis in knees with small effusions. The left thigh was swollen, warm, and tender to touch. The swelling was all around the thigh and extended on the lateral aspect from the left greater trochanter to 2 cm above the left knee. Movements of both hip prosthesis were limited, though the left was more so because of the thigh pain. The calves were not swollen or tender. Other systemic examination was unremarkable.

LABORATORY TESTS

Haemoglobin was 12.2 g, whole blood count 16.5, 81% neutrophils, platelets 422, erythrocyte sedimentation rate 56 mm/1st h (her usual ESR was around 25), asparate transaminase 78, alanine transaminase 38, and alkaline phosphatase 111 (normal values for AST 11-32, ALT 5-30, alkaline phosphatase 35-105). Urine analysis, blood urea nitrogen, creatinine, and serum bilirubin were normal and blood cultures were negative. x Rays of her left femur showed diffuse soft tissue swelling but no bony abnormalities. x Ray of the pelvis showed bilateral hip prosthesis with no radiological evidence of loosening or infection. Doppler ultrasound examination of the left lower limb showed patent deep veins and a large pocket of fluctuant fluid collection in deep thigh. Computed tomography of the left thigh (see figure 1) showed diffuse swelling, irregular contrast enhancement, and multiple low attenuation collections within the anterior compartment (vastus muscles). The left hip prosthesis was not involved in the pathological process. A diagnosis of pyomyositis was made. She was taken to the operating room for incision and drainage and pus was drained from the anterior and lateral compartments of the left thigh. Over the next week, she needed two more operations to drain the abscess cavities fully. Gram stain of the pus showed Gram positive cocci in clusters and the culture grew Peptostreptococcus sp, an anaerobic streptococcus, which is a normal commensal of the oral cavity. The patient denied any recent oral symptoms and stated that she had not seen a dentist in many years. A dental opinion was sought. Oral examination showed poor periodontal hygiene with receding gum margins, dental plaque, and caries in her five teeth. She did not have temporomandibular joint involvement and there was no evidence of oral candida. Subsequent radiographic examination showed multiple gingival abscesses, which needed surgical drainage. By this time the patient was already taking antibiotics and cultures of the gingival scrapings did not grow any pathogens.

Figure 1

Computed tomography of both thighs. The left thigh is swollen and shows subcutaneous oedema and loculated fluid collection (arrow).

Discussion

We have described a case of gingival abscess secondary to severe xerostomia, with a progression to low grade septicaemia and a pyomyositis.

Xerostomia or dry mouth is a common complaint in patients with secondary Sjögren’s syndrome and is seen in 43% of patients with primary Sjögren’s syndrome.1 Patients often report difficulty in swallowing dry food, inability to speak continuously, changes in sense of taste, and an increase in oral infections, especially dental caries and sialadinitis.2 Absence of saliva plays an important part in dental caries, periodontal disease, and removal of salivary glands in experimental animals significantly increases the incidence of dental caries,3 and periodontal disease.4

Salivary secretions are protective as their physiological role is to maintain the integrity of the oral tissues (see table 1). Saliva exerts an important influence on dental plaque by mechanically cleansing the exposed oral surfaces, by buffering acids produced by bacteria, and by controlling bacterial activity. Saliva contains numerous inorganic and organic factors that influence bacteria and their products in the oral environment. Inorganic factors include ions and gases, bicarbonate, sodium, potassium, phosphates, calcium, and fluoride. Organic factors include lysozyme, lactoferrin, myeloperoxidase, lactoperoxidase, agglutinins such as glycoproteins, and antibodies.5

Table 1

Role of saliva in oral health

Lysozyme is an enzyme in saliva that cleaves the linkage between structural components of the glycoprotein cell wall of both Gram positive and negative bacteria. It repels certain transient bacterial invaders of the mouth.6 Lactoferrin binds avidly to iron molecules, which are essential to the bacterial growth.7The lactoperoxidase-thiocyanate system in saliva has been shown to be bactericidal to some strains of Streptococcus andLactobacillus 8 by preventing accumulation of lysine and glutamic acid, both essential for bacterial growth. Myeloperoxidase is released by leucocytes and prevents attachment ofActinobaccillus to the dental enamel.9 It is also bactericidal. Saliva contains antibodies that are reactive against indigenous oral bacterial species. Although immunoglobulins IgG and IgM are found, the most preponderant immunoglobulin is IgA. IgA antibodies coat bacteria and prevent the attachment of oralStreptococcal sp to mucosal epithelial and dental surfaces.10

Proteolytic enzymes in saliva are generated by both, the host and oral bacteria. These enzymes have been recognised as contributors to the initiation and progression of periodontal disease. To combat these enzymes saliva contains antiproteases such as cathepsin and antileucoproteases.11 High molecular weight mucinous glycoproteins in saliva bind specifically to many plaque forming bacteria. They inhibit adsorption of some bacteria to the tooth surface and epithelial surfaces in the oral cavity.5

Patients with Sjögren’s syndrome can suffer rapid and devastating dental decay, which can lead to apical abscesses, gingival abscesses, and rapid loss of teeth. With the absence of saliva and its protective properties, patients with xerostomia need particularly scrupulous maintenance of oral hygiene. The artificial saliva preparations are useful only for lubrication purposes and do not have any antibacterial properties. However in patients with Sjögren’s syndrome secondary to rheumatoid disease, severe arthritis may cause functional impairment leading to practical difficulties in daily brushing and flossing.

We recommend the following measures to maintain oral hygiene in such patients:

(1) Rheumatologists should remind and re-enforce the importance of regular dental care at every consultation.

(2) Sugar free sweets should be used to improve salivation. Mouth washes with high sugar content or sucking of sugar containing sweets should be avoided.

(3) All patients should use antibacterial mouth washes (for example, chlorhexidine) before retiring at night and in addition, dentate patients should use fluoride mouth washes once daily.

(4) Twice a year visits to the dentist or dental hygienist are recommended.

The lesson

• Just as the rheumatologists send their patients who are taking hydroxychloroquine to the ophthalmologists for periodic eye check up, the Sjögren’s syndrome patients should be sent to the dentists twice a year for maintenance of oral hygiene.

References

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