Pierantonio Ostuni

Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren's Syndrome.

OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjögrens syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.

Hepatitis B reactivation in a chronic hepatitis B surface antigen carrier with rheumatoid arthritis treated with infliximab and low dose methotrexate

Infliximab, a humanised, mouse derived, genetically engineered, monoclonal antibody to tumour necrosis factor α (TNFα), is successfully used in association with low dose methotrexate in the treatment of rheumatoid arthritis (RA).1 Serious infections have been reported to be associated with infliximab treatment.1,2 However, the safety of infliximab is unknown or has not been yet established in chronic viral infections, including human immunodeficiency virus, hepatitis B virus (HBV) or hepatitis C virus infections. We describe a case, from our cohort of 102 patients treated with infliximab plus methotrexate, who carried hepatitis B surface antigen (HBsAg) and subsequently developed acute hepatitis due to HBV reactivation after 16 months of treatment with infliximab. A 49 year old man was diagnosed as having RA in January 1990. HBsAg, and HBe and HBc antibodies were positive, while HBe antigen and HBs …

Esophageal motor function in primary Sjögren's syndrome : Correlation with dysphagia and xerostomia

The incidence of dysphagia in patients withprimary Sjögrens syndrome (pSS) has beenunderestimated and all too often ascribed to xerostomia,without considering the possible presence of esophagealmotor abnormalities affecting other nonsclerodermaconnective tissue diseases. Esophageal and salivaryfunctions were prospectively evaluated in 27 females whomet the four criteria proposed by Fox for the diagnosis of pSS, using esophageal manometry after wetswallows and Saxons test, respectively. Dysphagia wasgraded using a standard symptoms questionnaire andresults were compared with those obtained in a group of 21 healthy controls. Seven patients with pSS(26%) had no swallowing discomfort, 2 (7.4%) had milddysphagia, 7 (26%) had moderate dysphagia, and 11(40.6%) had severe dysphagia. Saxons test revealed an overall decrease in the salivary flow ratecompared to controls, with no difference betweenpatients with or without dysphagia. Esophageal manometrydemonstrated the absence of any lower or upperesophageal sphincter function abnormalities in allpatients. In the patients with pSS as a whole,manometric study of the esophageal body showed a motorpattern comparable with that of controls, with nodifference between patients with and without dysphagia.Defective peristalsis, ie, the presence of simultaneouscontractions in more than 30% of wet swallows wasdetected, however, in the distal tract of the esophagus of six patients (22.2%) and in the proximaltract of three (11.1%). All these patients had severedysphagia and the modified Saxons test revealed asalivary secretion comparable with that of patients with a normal peristalsis. Dysphagia is a verycommon complaint in patients with pSS and does not seemto correlate with xerostomia, which is a constant andtypical finding of the disease. About one third of patients with pSS have an abnormal esophagealperistalsis that is responsible for severe dysphagia,whereas decreased salivary outflow exacerbates theswallowing discomfort. This has to be taken into account and justifies the routine use of esophagealmanometry in patients with pSS. The cause of dysphagiain pSS patients without peristaltic disorders of theesophagus has to be investigated.

Fibromyalgia in Italian patients with primary Sjögren's syndrome

OBJECTIVE To assess the prevalence of fibromyalgia in primary Sjögrens syndrome and to evaluate the clinical differences between patients affected with both primary fibromyalgia and primary Sjögrens syndrome and those affected only with primary fibromyalgia. METHODS Clinical features of fibromyalgia were evaluated in 100 consecutive outpatients with primary Sjögrens syndrome and, as controls, in 90 patients with non-insulin-dependent diabetes mellitus, in 75 patients with primary fibromyalgia and in 30 healthy subjects. RESULTS Fibromyalgia was recorded in 22% of patients with primary Sjögrens syndrome, in 12.2% with diabetes and in 3.3% of healthy controls. In the primary Sjögrens syndrome group the prevalence was significantly higher than in healthy controls (P < 0.01), but not significantly different than in diabetes. Moreover, primary Sjögrens syndrome with fibromyalgia and primary fibromyalgia patients did not differ with respect to the number of tender points, while the mean pain threshold was lower in the latter (P = 0.05). Purpura, hypergammaglobulinemia, rheumatoid factor, and a focus score > or = 1 on lip biopsy were significantly more frequent in primary Sjögrens syndrome patients without than with fibromyalgia. CONCLUSIONS As recently reported by other authors, our study confirms the moderate increase of fibromyalgia prevalence in primary Sjögrens syndrome. Typical fibromyalgic findings are quite similar to those of primary fibromyalgia, but surprisingly, primary Sjögrens syndrome patients with fibromyalgia show a less severe global involvement than those with primary Sjögrens syndrome alone.

Elderly onset of primary Sjögren’s syndrome: Clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients

OBJECTIVES To study and compare the clinical and serological features of patients with elderly versus adult and younger onset of primary Sjögrens syndrome (pSS). METHODS We analyzed retrospectively 336 consecutive pSS patients followed at our unit. They were subdivided into three groups according to the age at disease onset: elderly (>65 years), adult (>40 and ≤65 years), and young (≤40 years). Clinical and immunological features of the disease, labial salivary glands biopsy, ocular and oral tests were collected at time of diagnosis and then compared among the three groups. RESULTS In 21 (6%) patients, disease onset occurred after the age of 65 years. At the time of diagnosis, 15 (71.4%) of these patients reported symptoms of dry mouth and 16 (76.1%) of dry eye. The most common extraglandular manifestation were arthralgias in 14 (66.7%), Raynauds phenomenon in five (23.8%) and purpura in three (14.2%) cases. Ocular diagnostic tests (Schirmers I and Rose-Bengal staining) were positive respectively in 17 (80%) and nine (44.4%) patients. In eight (38%) cases, unstimulated whole salivary flow showed normal values, while 12 patients (57.1%) showed positivity for salivary sialography. A focus score greater or equal to 1 per 4mm(2) was demonstrated in 11 (53.3%) of the 21 cases. CONCLUSION Elderly onset of pSS was associated with similar incidence of the diagnostic tests positivity (parotid sialography, ocular tests, minor salivary gland biopsy) in comparison with adult and younger onset. Moreover, no statistical differences were found among the three groups concerning sex, disease duration, as well as ocular and oral symptoms.

Serum and salivary neopterin and interferon‐γ in primary Sjögren's syndromeCorrelation with clinical, laboratory and histopathologic features

Objective: To investigate serum and salivary neopterin and interferon‐γ as possible markers of immune system activation in primary Sjögrens Syndrome (pSS). Methods: Serum and salivary neopterin and interferon‐γ concentrations were determined in 30 untreated patients with pSS and matched with several other clinical and laboratory parameters. Results: The mean concentration of neopterin was significantly higher in pSS patients (8.12±3.36 nmol/L in serum and 9.50±7.61 nmol/L in saliva) than in normal controls (p<0.05). Significant correlations were found between serum neopterin and β2‐microglobulin, serum IgG as well as lip biopsy score. Salivary neopterin concentration was inversely related to Shirmer‐I test, tear break‐up time and stimulated salivary flow rate. Serum and salivary levels of interferon‐γ were normal and no correlation with the other parameters was found. Conclusion: In pSS patients serum neopterin may represent a useful marker of cell‐mediated immunity. On the other hand, salivary neopterin seems to reflect the glandular damage.

Dermatomyositis and malignancy of the pharynx in Caucasian patients: report of two observations.

The association between dermatomyositis and malignancy of the pharynx is rare among whites but not uncommon among Far Eastern and north African populations. We report two cases of Caucasian Italian patients with dermatomyositis associated, respectively, with nasopharyngeal and tonsillar carcinomas. The relationship between dermatomyositis and malignancy is also discussed.

Hyper-IGD syndrome: A new case treated with colchicine

SummaryWe report a new case of hyper-IgD syndrome, a recently described disease characterized by recurrent episodes of fever with headache, bilateral cervical lymphadenopathy and, more rarely, abdominal pain and diarrhoea. Polyclonal increase of serum IgD is the most important laboratory finding. Etiopathogenesis and differences with familial Mediterranean fever are discussed. Moreover, good results obtained with colchicine treatment are also reported.

Atypical hypergammaglobulinaemia D syndrome with amyloidosis: an overlap with familial Mediterranean fever?

SummaryA case of hypergammaglobulinaemia D and periodic fever syndrome, developing an amyloidosis-related nephrotic syndrome, is reported. Since such a complication represents a typical feature of another disease characterized by recurrent febrile attacks, i.e., familial Mediterranean fever, an overlap syndrome between these two rare clinical disorders can be suggested.

Adult Henoch–Schönlein Purpura with Glomerulonephritis and Paroxysmal Nocturnal Haemoglobinuria: An Uncommon Association

Abstract: Henoch–Schönlein purpura (HSP) is considered to be a small blood vessel systemic vasculitis. Numerous microorganisms have been implicated in triggering HSP. We describe an unusual case of HSP with glomerulonephritis and renal failure requiring haemodialysis in a young adult man who subsequently developed paroxysmal nocturnal haemoglobinuria (PNH) with several haemolytic episodes. Bacterial infections, especially those of the respiratory and urinary tract, might trigger both the diseases.