Rare Presentation of a Metastatic Pancreatic Neuroendocrine Neoplasm Presenting with Atrial Flutter.

Pancreatic neuroendocrine neoplasms (PanNENs) rarely secrete serotonin, which is the main cause of carcinoid syndrome. One of its unusual manifestations is carcinoid heart disease or Hedinger's syndrome which is seldom accompanied by cardiac arrhythmias. We report the case of an 88-year-old woman who presented with recently experienced episodes of palpitations and a newly developed atrial flutter with a ventricular rate of 130 beats per minute. Echocardiography revealed thickened and tethered tricuspid and pulmonary valve leaflets causing severe valvular regurgitation and right ventricular dilatation. Episodes of intermittent diarrhoea over the previous 2 years were mentioned, making carcinoid syndrome our working diagnosis. The 5-hydroxyindoleacetic acid (5-HIAA) levels in a 24-hour urine collection specimen were elevated. Conventional imaging studies and a Ga-68 dodecane tetraacetic acid tyrosine-3-octreotate (DOTATATE) positron emission tomography/computer tomography (PET/CT) scan revealed the presence of a metastatic PanNEN arising from the pancreatic tail. The patient was managed with lanreotide and telotristat with remarkable improvement of her symptoms. To our knowledge, this is the first reported case of carcinoid syndrome presenting with atrial flutter as the initial symptom. LEARNING POINTS: Ultrasonography findings can indicate or lead to the diagnosis of carcinoid heart disease or Hedinger's syndrome.Clinicians should investigate rarer causes of atrial flutter when common ones are excluded.Even in advanced metastatic disease, complete remission of symptoms may be achieved with somatostatin analogues along with telotristat ethyl.

Early rheumatoid arthritis patients: relationship of age.

The aim of this study was to investigate if age at disease onset comprises a separate parameter for disease expression, prognosis, and outcome in early rheumatoid arthritis (RA) patients. Four hundred thirty-eight patients with early RA (disease duration less than 1 year) were studied. All of them fulfilled the American College of Rheumatology criteria for RA. The demographic, clinical, laboratory, radiologic, and therapeutic characteristics of the disease at diagnosis and during and at the end of follow-up (time period 1981-2000) were analyzed according to age at disease onset (young patients aged less than 60 years at disease onset vs elderly patients aged more than 60 years at disease onset). We found 317 young and 121 elderly patients with early RA. The male:female ratio, which was 1:3.2 in the young patients, was nearly equal in the elderly (1:1.4). In addition, at disease onset elderly patients showed more severe joint involvement (decreased grip strength) associated with high titers of acute phase response (erythrocyte sedimentation rate and C-reactive protein) than the younger patients. However, there were no differences between the two groups in the numbers of tender and swollen joints or acute phase response at the end of the study period. Furthermore, no differences were seen between the two groups concerning the presence of rheumatoid factor. Finally, the two patient groups showed the same degree of radiological changes and functional ability and were treated similarly, except for more frequent corticosteroid use in the elderly. We conclude that elderly patients present with more severe joint involvement at disease onset. However, at the end of the study, no differences were seen concerning radiological changes and functional ability. It seems that age at disease onset does not influence the clinical course and outcome of early RA patients.

Shared epitopes and rheumatoid arthritis: disease associations in Greece and meta-analysis of Mediterranean European populations.

OBJECTIVES: To assess the strength of the associations between HLA shared epitopes (SE) and rheumatoid arthritis (RA) susceptibility, articular disease severity, and extra-articular features in Mediterranean European populations. METHODS: One hundred and seventy-four Greek RA patients and 103 controls were evaluated. Data were then included in a meta-analysis of 9 studies of Mediterranean European populations (959 RA patients and 1,405 controls). RESULTS: In our study population, SE alleles were significantly more common in RA patients than in controls (odds ratio [OR], 2.5; 95% confidence interval [CI], 1.4-4.3). Larsen radiologic score was predicted by SE and disease duration. SE did not increase the risk of any extra-articular manifestation. The meta-analysis showed a pooled OR of 3.7 (95% CI, 2.6-5.2) for susceptibility to RA conferred by SE (OR, 3.4 v 3.9 in Greek v non-Greek populations). CONCLUSIONS: SE determine articular destruction without increasing the risk of extra-articular manifestations. The immunogenetic associations of RA susceptibility are consistent, but their strength may depend on the SE prevalence in different ethnic groups.

Disease modifying antirheumatic drugs in early rheumatoid arthritis: a longterm observational study.

OBJECTIVE: To investigate the effectiveness, toxicity, and drug survival in an observational longterm study of patients with early rheumatoid arthritis (RA). METHODS: Four hundred twenty-eight patients with early RA were investigated between January 1987 and December 1995. All patients had a disease duration of less than one year and had not been previously treated with any disease modifying antirheumatic drug (DMARD). The following drugs were introduced at the doses specified: hydroxychloroquine (HCQ) (200-400 mg/day), D-penicillamine (D-Pen) (500 mg/day), sulfasalazine (SSZ) (2-3 g/day), auranofin (6 mg/day), intramuscular gold (IM gold, 50 mg/week), methotrexate (MTX) (0.15 mg/kg/week, per os), cyclosporin A (CSA) (3 mg/kg/day), azathioprine (AZA) (2-3 mg/kg/day), cyclophosphamide (CYC) (1-2 mg/kg/day). RESULTS: Three hundred eighty-three patients were treated with one DMARD for at least 6 months. Sixty-five percent of patients were seropositive. The disease duration was 9.2 (3.1) months and the followup period of 12.7 (4.8) years, ranging from 7 months to 13 years. The drugs of first choice were: D-Pen (32%), HCQ (30%), MTX (21%), CSA (8%), and IM gold (7%). After the 2nd, 3rd, and 4th prescriptions, MTX was the most popular drug (27%), while D-Pen and HCQ were prescribed less frequently. The longest drug survival was seen in MTX treated patients, followed by CSA, without significant differences between them. D-Pen, HCQ, and IM gold had the largest dropout rate. The main causes for drug discontinuation were drug inefficacy (HCQ), followed by adverse drug reactions (D-Pen). CONCLUSION: It appears that MTX has the longest survival time, with CSA following in second place. The main reasons for discontinuation of treatment were drug inefficacy, followed by adverse drug reactions.