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Raynaud's phenomenon (RP) is rare in young children. We describe two infants with severe RP, manifesting as fingertip necrosis, who were resistant to conventional vasodilators and were treated successfully with iloprost, a prostacyclin analogue. The application of iloprost is safe and should be considered in children with threatening ischemic digits.

Objective: A large proportion of patients with limited systemic sclerosis (SSc) do not meet the current American College of Rheumatology (ACR) classification criteria for SSc. We undertook this study to determine whether the addition of easily available clinical variables, namely nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias, could improve the sensitivity of the current ACR classification criteria for patients with limited SSc., Methods: Patients in the Canadian Scleroderma Research Group Registry with skin involvement distal to the metacarpophalangeal joints were identified and divided into two groups according to whether they fulfilled the current ACR classification criteria for SSc or not. Sensitivity of the criteria was calculated. Regression tree analysis was performed to determine whether the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias could improve the sensitivity of the criteria., Results: One hundred and one (101) patients were included, in majority women with a mean age of 59 (+/- 13). Of these, 68 (67%) met the ACR classification criteria. The sensitivity of the criteria increased from 67% to 99% with the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias., Conclusions: The SSc research community would benefit from having better classification criteria to identify patients with limited SSc. The current classification criteria for SSc may be significantly improved by the inclusion of easily identified clinical variables including nailfold capillary abnormalities using a dermatoscope.

Objective: Presence of extra-articular manifestations (EAM) in rheumatoid arthritis (RA) is associated with more severe disease and increased mortality. Prevalence of EAM may vary in different geographic areas and in different ethnic populations. In this study we investigated the frequency of EAM in 526 RA patients from a single university hospital in Turkey., Methods: The hospital records of patients who had been diagnosed as RA in Hacettepe University Department of Rheumatology between the years 1988 and 2003 were retrospectively evaluated. There were 73 males and 453 females, and mean age of the patients was 48.0 +/- 12.3 years. The mean follow-up period was 4.8 +/- 4.1 years. Three hundred and fifty-nine patients were rheumatoid factor (RF) positive (68.3%)., Results: The overall frequency of EAM was 38.4% (202 patients). The most common EAM was rheumatoid nodules (18.1%). Sicca symptoms, pulmonary findings, Raynaud's phenomenon, livedo reticularis, carpal tunnel syndrome, vasculitis, amyloidosis, and Felty syndrome were present in 11.4%, 4.8%, 3%, 4.8%, 2.8%, 1.3%, 1.1%, and 0.3% of the patients, respectively. Overall EAM and rheumatoid nodules were significantly more common in RF positive patients than RF negative patients. The frequency of rheumatoid nodules was significantly higher in males than in females., Conclusion: The prevalence of EAM in Turkey is higher than East Asia and Africa, and lower than UK and North America. Excluding secondary Sjögren's syndrome, our results are similar to other Mediterranean populations like Italy.

Objective: Upper extremity angiography can make an important contribution to the diagnosis in vasculopathy. The present study was designed to assess the diagnostic role of upper extremity angiography in patients with disturbed circulation of the hand, according to a standardised protocol., Methods: The study was carried out in an outpatient setting in 103 patients suffering from bilateral Raynaud's phenomenon without any obvious underlying disease and who were unresponsive to nifedipine and aspirin. All patients had angiographies taken according to a standardized technique using vasodilating medication, and reviewed according to a standardised protocol that covered all the known characteristics of angiopathy such as diminished flow, stops, tortuosity, irregularity of the wall, tapering, collaterals and blushing., Results: Standardised angiograms showed vasculopathy compatible with primary vasospasm in 42 patients [all women; mean age 35.1 years], atherosclerotic vascular disease in 44 patients [M/F 9/35; mean age 46.7 years], peripheral embolism in 8 patients [M/F 4/4; mean age 38.4 years], vasculitis in 3 patients [3 women; mean age 38 years] and Buerger's disease in 3 patients [3 men; mean age 47 years]. Inter-observer differences were present in 4 cases, but consensus could be reached through open discussion. An unexpected 47% of patients with atherosclerotic vascular disease had dyslipidemia, frequently of familial origin., Conclusions: The standardised angiography protocol proved to be helpful in the assessment of upper extremity angiography. Surprisingly, a high prevalence of angiographic abnormalities compatible with atherosclerotic vascular disease could already be diagnosed in relatively young patients with Raynaud's phenomenon, of whom 47% showed hypercholesterolemia.

Overt pulmonary hypertension in systemic lupus erythematosus (SLE) is rarely reported. We have documented 4 such patients, all of whom had severe extrapulmonary manifestations of the disease and also Raynaud's phenomenon. The relationship between Raynaud's phenomenon and pulmonary hypertension in SLE is borne out by a review of the literature. A common vasospastic aetiology is suggested.

A patient with pulmonary hypertension (PH) complicating mixed connective tissue disease (MCTD) is described. The patient died suddenly after catheterization documenting the pulmonary hypertension. Pathologic examination demonstrated marked medial hypertrophy and intimal proliferation of the pulmonary arterial vessels. Review of the literature revealed few and differing reports concerning the pathology of PH as seen in MCTD. Sudden death in patients with pulmonary hypertension undergoing invasive procedures is not an uncommon occurrence.

The bronchoalveolar lavage (BAL) technique was used to characterize inflammatory cells in the lower respiratory tract of seven nonsmoking patients with CREST syndrome and interstitial pulmonary fibrosis. Differential cell counts in the BAL fluid showed a significant increase of neutrophils (p less than 0.05) and eosinophils (p less than 0.01) in comparison to normal subjects. In three patients there was also an increase of lymphocytes. No correlation was found between BAL and duration of scleroderma and/or lung function tests. The BAL findings in these scleroderma patients resemble those of idiopathic interstitial fibrosis suggesting that a similar inflammatory process occurs within the lower respiratory tract.

Various pleuropulmonary manifestations are reported to occur in the course of systemic lupus erythematosus (SLE). Among these, pulmonary hypertension is one of the most uncommonly observed. The natural course of this involvement can be independent of the primary disease, and resembles primitive pulmonary hypertension. Various pathogenetic mechanisms might be responsible for this picture. In the case here reported, the pathological data were strongly suggestive of pulmonary vasculitis, emphasizing that SLE, like other connective tissue diseases, can be responsible for fatal pulmonary hypertension.

In vivo capillary examination was performed on 80 patients included in a prospective study of Raynaud phenomenon (RP): 40 RP only (RPO), 20 undifferentiated connective tissue disease (UCTD) and 20 systemic scleroderma (SD) of less than 5 years' duration. On initial examination, SD-pattern capillary abnormalities were found in 7/40 patients with RP alone, 18/20 with UCTD and 19/20 with SD. On follow-up a change of diagnosis into a definite connective tissue disease occurred in 4 patients with UCTD; one patient with RP alone died with symptoms of myositis and fulminant pulmonary vasculitis. All five showed SD-pattern capillary abnormalities of the "active" type on initial examination. A significant correlation between the "slow" capillary pattern and the presence of anticentromere antibodies was also observed. Only one patient of 25 RPO with normal capillaries available for follow-up showed any change in clinical status.