Pericardial effusion and giant cell arteritis.

Pericardial effusion in patients with giant cell arteritis has been described in case reports. The exact prevalence of this manifestation is unknown. The objective of this study was to determine the prevalence and characteristics of pericardial effusion in patients with giant cell arteritis. Retrospective data were collected from 114 patients at a single hospital with giant cell arteritis diagnosed between October 1999 and July 2013. Pericardial effusion was found in four patients (3.5 %, 95 % confidence intervals 1-8.7). In three cases, pericardial effusion was present at initial presentation and was asymptomatic. In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced. Prognosis was good in all cases. The present study confirms that pericardial effusion could occur in patients with giant cell arteritis. However, pericardial effusion is a rare manifestation of giant cell arteritis. Clinical course of pericardial effusion in the setting of giant cell arteritis is usually benign and rarely symptomatic. Recognition of this manifestation may contribute to early diagnosis of the disease.

Dry cough is a frequent manifestation of giant cell arteritis.

Physicians need to be familiar with the typical manifestations of giant cell arteritis. However, the challenge lies in recognizing atypical cases that lack the more specific manifestations or reflect vasculitis in less frequently involved territories. Among atypical clinical manifestations, dry cough has been reported in recent years. The literature contains sporadic reports mainly single case report. The objective of this study was to determine the frequency of dry cough in patients with giant cell arteritis. Clinical data were collected from 88 patients with giant cell arteritis. Relationships between dry cough and other clinical manifestations or biological data were analyzed. Dry cough of recent appearance was found at initial presentation of giant cell arteritis in 12 patients (13.6%). In 2 cases, dry cough was isolated. The 2 patients sought attention because of chronic dry cough associated with inflammation of unknown origin. In 10 cases, dry cough was associated with typical clinical manifestations of giant cell arteritis. A correlation was found between inflammatory biomarkers and presence of dry cough. The mean CRP was 153.8 mg/l (SD 85.1) in patients with dry cough and 94 mg/l (SD 72.2) in patients without dry cough (p = 0.0131). We conclude that the diagnosis of giant cell arteritis should always be considered in an elderly patient with an unexplained elevation of inflammatory markers and chronic dry cough. Dry cough in giant cell arteritis was not correlated with other clinical manifestations of this vasculitis, including pulmonary manifestations, but was correlated with inflammatory biomarkers.

Characteristics of cerebrovascular accidents at time of diagnosis in a series of 98 patients with giant cell arteritis.

The objective of this study was to determine the characteristics of cerebrovascular accidents at time of diagnosis in patients with giant cell arteritis. Retrospective data were collected from 98 patients at a single hospital with giant cell arteritis (according to the American College of Rheumatology classification criteria) diagnosed between October 1999 and January 2012. Cerebrovascular accident was found at initial presentation in 6 patients (6.1 %, 95 % CIs 2.3-12.9). Most of them had other symptoms of giant cell arteritis when the disease began. Signs reflecting the involvement of vertebro-basilar territory were present in 3 cases. No other case of cerebrovascular accident was described during the follow-up of patient; particularly no case of cerebrovascular accident occurred once corticosteroid therapy for the treatment of giant cell arteritis had been initiated. No differences in the epidemiologic, clinical and laboratory features at the time of diagnosis between patients who had cerebrovascular accidents and the rest of the giant cell arteritis patients were observed. Prognosis was good in our survey. However, there was no case of bilateral vertebral artery occlusion, a condition associated with poor prognosis. The present study confirms that cerebrovascular accidents may be the initial manifestation of giant cell arteritis, an argument in favor of a direct effect of the vasculitis in the development of cerebrovascular accidents rather than a complication of the corticosteroid therapy. The diagnosis of giant cell arteritis should always be considered in an elderly patient with stroke and an unexplained elevation of inflammatory biomarkers.

Camptocormia as a clinical manifestation of polymyositis/systemic sclerosis overlap myositis associated with anti-Ku.

Camptocormia, an abnormal truncal flexion posture that occurs while walking or standing, is usually caused by various hypokinetic movement disorders, mainly Parkinson disease. We describe the case of a man with subacute onset of camptocormia. Quadriceps muscle biopsy showed significant rhabdomyolysis, few isolated inflammatory cells and mild expression of type I MHC in few fibers, a pattern usually found in immune-mediated necrotizing myopathies. Myositis was associated with Raynaud's phenomenon, mild sclerodactyly, and anti-Ku antibodies leading to the diagnosis of polymyositis/systemic sclerosis overlap myositis. The posture showed modest improvement in response to treatment.

Systemic mastocytosis: predictable factors of poor prognosis present at the onset of the disease.

Systemic mastocytosis (SM) refers to a group of heterogeneous diseases that can be divided into indolent SM, for which prognosis is favorable, and malignant SM, which has a poor prognosis. While the diagnosis of SM is often a challenge since clinical and biological abnormalities are not specific, prognosis is even more difficult to predict. Thus, we aimed to highlight predictable factors in a cohort of 28 cases of SM. Among the 13 women and 15 men studied were 7 patients who had an aggressive form of SM that ultimately led to death in 3 of them. We found common characteristics among these seven patients. First, they were older when the first symptoms appeared and when the diagnosis was confirmed. Second, ascitis, lymphadenopathy, anemia, and thrombocytopenia were significantly more frequent, while cutaneous lesions and flush were less frequent. Moreover, general symptoms, gastrointestinal disorders, neutropenia, and coagulation abnormalities also seemed to characterize this group of patients. Understanding the factors that predict SM is essential in order to provide patients with the malignant form of the disease with specific treatments.

Longitudinal study of oxidative status in 312 cystic fibrosis patients in stable state and during bronchial exacerbation.

In cystic fibrosis (CF), there is an imbalance in the oxidant/antioxidant system, leading to oxidative damage. The aim of this study was to assess antioxidant-scavenger deficiencies and lipid peroxide variations in three clinical situations (stable status, acute exacerbation, and after intravenous antibiotic treatment). The objective was also to correlate oxidative stress with age, nutritional status, and respiratory function. The study included prospectively 312 consecutive patients and 53 controls. Antioxidants (vitamin A, vitamin E, carotenoids, and glutathione) and oxidative markers (malondialdehyde and lipid peroxides) were measured in plasma. Regression analyses were performed. Antioxidant levels were lower in CF patients than in controls. These levels decreased during acute exacerbation and increased after antibiotic treatment. Carotenoid levels were not modified by infection or age. Only vitamin A and carotenoid levels were positively correlated to body mass index. Antioxidant levels were correlated to forced expiratory volume at 1 sec. Lipid peroxidation markers were lower in patients than in controls. Their levels decreased during infection, and increased after antibiotic treatment. Impaired lung function was correlated with elevated malondialdehyde levels. In conclusion, this study demonstrates antioxidant deficiency in a very large cohort of CF patients. Carotenoid and vitamin E deficiencies occur early in the course of the disease. Antioxidants decrease with bronchial infection. By contrast, nutritional disorders did not modify antioxidant levels during acute exacerbations. Thus, pulmonary disorders rather than nutritional disorders seem to be essential in the imbalance of the oxidant/antioxidant system. Results concerning glutathione and oxidative-marker levels highlighted the fact that their plasma values do not reflect oxidative stress in the respiratory tract.

Whipple's disease with muscle impairment.

A 67-year-old man presented with myalgia, muscle atrophy, and a history of seronegative polyarthritis. Blood tests showed inflammation but no hematologic or immunologic abnormalities. Muscle biopsy revealed no vasculitis or myositis but Tropheryma whipplei was detected by polymerase chain reaction in muscle, blood, and duodenum specimens; this was confirmed by immunohistochemistry. Ceftriaxone led to clinical improvement. Although rare, Whipple's disease should be considered in the differential diagnosis of diffuse myopathy.