Relation of gelsolin amyloidosis and periodontal health.

OBJECTIVE: Hereditary gelsolin amyloidosis (AGel amyloidosis) is a rare, dominantly inherited systemic disease with worldwide distribution, caused by a gelsolin gene mutation. We studied the periodontal conditions and microbiological plaque composition of AGel amyloidosis patients. MATERIAL AND METHODS: A voluntary study group of 36 AGel amyloidosis patients (mean age 61) filled in a questionnaire. A thorough periodontal examination included periodontal pocket depth and attachment level measurements, registrations of visible plaque, bleeding on probing and panoramic radiographs. The presence of oral Candida was studied by fungal culture method. Bacterial samples from deepened pockets (≥4 mm) were analyzed with checkerboard DNA-DNA hybridization method. RESULTS: VPI (15.3 %) and BOP (11.2 %) of the patients were modest reflecting relatively adequate oral self-care. Still 89 % of the patients had at least one PPD of ≥4 mm; 78.5 % of the PPDs ≥6 mm were found in molars. Patients had lost one third of the molars due to periodontitis and/or tooth decay. Half of the patients (53 %) were Candida carriers. Bacterial analysis of subgingival plaque samples revealed bacterial species common to chronic periodontitis. CONCLUSION: AGel amyloidosis may increase the risk for periodontitis even when the oral self-care is adequate. Molar teeth appear to be mostly affected, leading to tooth loss. CLINICAL RELEVANCE: AGel amyloidosis as a systemic disease is related with a vast variety of symptoms with variable severity. Even though a causal relationship of the systemic disease and periodontitis has not yet been proven, increased risk for periodontal problems should be considered when examining AGel amyloidosis patients.

Xerostomia in hereditary gelsolin amyloidosis.

Hereditary gelsolin amyloidosis (AGel amyloidosis) is a rare, dominantly inherited systemic disease with worldwide distribution, caused by c.654G > A or c.654G > T gelsolin gene mutation. The disease mainly manifests with late-onset dystrophy of the cornea, laxity of the skin and dysfunction of the cranial nerves whereas the oral manifestations have remained less-studied. To examine if AGel amyloidosis also affects salivary gland function, we studied 27 patients. In a questionnaire, 89% of them reported oral dryness, and 74% oral and ocular dryness. Unstimulated (UWS) and stimulated whole salivary flow (SWS) rates were measured, and salivary proteins were analyzed in the patients and controls. Hyposalivation according to UWS was detected in 67% of the patients, while decreased SWS occurred in 63% of the patients and 19% of the controls (p = 0.001). The secretion rates of salivary total protein and IgA were significantly lower in patients than controls. Histopathological analyses of labial salivary gland biopsies showed deposition of gelsolin amyloid, atrophy and inflammation. This study showed that AGel amyloidosis belongs to the differential diagnostic choices to be kept in mind in the patients presenting with xerostomia, low secretion rates of salivary total protein and IgA and/or deposition of amyloid in the minor salivary glands. AGel amyloidosis patients should be advised for efficient dental care.

Hereditary gelsolin amyloidosis mimicking Sjögren's syndrome.

Hereditary gelsolin amyloidosis (AGel amyloidosis) belongs to the wide group of amyloidotic diseases, which comprise various hereditary but also sporadic forms, such as inflammation-associated AA amyloidosis, primary or myeloma-associated AL amyloidosis and common Alzheimer's disease and type II diabetes-associated local amyloidoses. AGel amyloidosis caused by a gelsolin G654A gene mutation is autosomally dominantly inherited and presents typically in the 30s with progressive corneal lattice dystrophy, followed by cutis laxa and cranial polyneuropathy. Here, we present a case of sicca syndrome, originally diagnosed as primary Sjögren's syndrome (SS) but later found to represent an initial disease manifestation of AGel amyloidosis, not recognised earlier. This case emphasises both the importance of specific amyloid stainings and comprehensive salivary gland histopathology as well as family history in SS differential diagnostics.

Periodontal disease and bacterial vaginosis increase the risk for adverse pregnancy outcome.

OBJECTIVES: To determine whether periodontal disease or bacterial vaginosis (BV) diagnosed before pregnancy increase the risk for adverse pregnancy outcome. METHODS: We enrolled a total of 252 women who had discontinued contraception in order to become pregnant. The first 130 pregnant women were included in the analyses. RESULTS: Multivariate analysis showed a strong association between periodontal disease and adverse pregnancy outcome (OR 5.5, 95% confidence interval 1.4-21.2; p = 0.014), and a borderline association between BV and adverse pregnancy outcome (OR 3.2, 95% confidence interval 0.9-10.7; p = 0.061). CONCLUSION: Our study suggests that pre-pregnancy counseling should include both oral and vaginal examinations to rule out periodontal disease and BV. This may ultimately have an impact on antenatal healthcare, and decrease the risk for adverse pregnancy outcome.