Role for vascular investigations in giant cell arteritis.

Giant cell arteritis is characterized by diffuse arterial inflammation that selectively involves the superficial temporal arteries but can occur in larger arteries. Various vascular investigations can assist in diagnosing and evaluating the extent of giant cell arteritis. Imaging techniques, mainly Doppler ultrasonography of the superficial temporal arteries, seem less reliable for the diagnosis than temporal artery biopsy, which is safe and remains indispensable. Investigations of larger arteries can detect asymptomatic stenotic lesions, which are common, particularly in the axillary and subclavian arteries. Involvement of the aorta can cause life-threatening dissection or aneurysmal rupture. Imaging techniques useful for diagnosing aortic involvement include ultrasonography, computed tomography, magnetic resonance imaging, and aortography. Although there is no standardized strategy for aortic lesion detection, helical computed tomography may be valuable.

[Digestive involvement of scleroderma]. / Atteintes digestives sclérodermiques.

Digestive involvement in systemic sclerosis is frequent and serious, because it provides morbidity and fatality. From the pathophysiologic point of view, the first step could be Raynaud-associated neural dysfunction, followed by smooth muscle atrophy then irreversible muscle fibrosis. Oesophageal disorder is common with its main consequence: the occurrence of gastroesophageal reflux disease which could run into peptic erosive oesophagitis. Oesophageal manometry is the main diagnostic tool, gastrointestinal endoscopy helps to assess oesophageal mucosal inflammation and its possible sequels. Gastric involvement is rarely recognized but it is frequent in case of systematic investigation as well as small intestinal involvement which may provide a lot of complications: malabsorption, pseudoobstruction, bacterial overgrowth. At colonic level, anorectal involvement is frequent and leads to fecal incontinence and rectal prolapse. Reynold's syndrome is a special case which associates systemic sclerosis with primary biliary cirrhosis. The symptomatic treatments must be systematic and improve the disease's overall prognosis.

First isolation of Tropheryma whipplei from bronchoalveolar fluid and clinical implications.

A patient presented diffuse pulmonary parenchymal micronodules. Tropheryma whipplei was detected in the saliva, a bronchial biopsy and bronchoalveolar fluid. PAS staining, immunohistochemistry and PCR for T. whipplei were negative in the duodenal biopsies. T. whipplei was isolated from the bronchoalveolar fluid, reinforcing its role as a respiratory pathogen.

Giant cell arteritis with or without aortitis at diagnosis. A retrospective study of 22 patients with longterm followup.

OBJECTIVE: Studies have shown that aortitis may be present in half the patients with recent-onset giant cell arteritis (GCA). We assessed whether aortitis at diagnosis affects longterm outcome in patients with GCA. METHODS: We retrospectively analyzed the longterm outcome of a prospective cohort of 22 patients with biopsy-proven GCA who all had aortic computed tomography (CT) evaluation at the time of diagnosis of GCA between May 1998 and November 1999. Longterm outcome, especially vascular events such as aortic aneurysm, mortality, relapses of GCA, and requirement for steroids, was assessed in 2011 by chart review and patient/physician interviews. RESULTS: At disease onset, 10/22 patients had aortitis on CT scan. Patients with and without aortitis had similar baseline characteristics, including cardiovascular risk profile. At the time of the study, 12/22 (57%) patients had died. Vascular causes of death were more frequent in patients with aortitis (5/7 vs 0/5; p = 0.02). A higher number of vascular events was noted in patients with aortitis (mean events per patient 1.33 vs 0.25; p = 0.009). Stroke was more frequent in patients with aortitis. These patients seemed to exhibit a more chronic or relapsing disease course, and they were less likely to completely discontinue steroid therapy (p = 0.009, log-rank test). CONCLUSION: Our study suggests for the first time that inflammatory aortic involvement present at onset of GCA could predict a more chronic/relapsing course of GCA, with higher steroid requirements and an increased risk for vascular events in the long term.

Paraneoplastic cutaneous leukocytoclastic vasculitis disclosing multiple myeloma: a case report.

Cutaneous lesions are unusual during the course of multiple myeloma. In rare cases, multiple myeloma may be associated to skin involvement secondary to amyloidosis, cryoglobulinemia, and POEMS syndrome. Paraneoplastic skin involvement occurs preferentially during solid neoplasms. We report the case of an uncommon presentation of an IgA lambda multiple myeloma in a 58 year-old woman preceded by vascular purpura with cutaneous leukocytoclastic vasculitis (LV) and intense deposit of IgA and kappa light chains in the dermal vessels. Purpura resolved after specific treatment of multiple myeloma and diagnosis of paraneoplastic purpura was asserted. We propose a brief review of the literature about skin involvement during multiple myeloma. This case highlights the necessity to search for MM when we are facing a LV.

Use of muscle biopsies for diagnosis of systemic vasculitides.

OBJECTIVE: Few studies have investigated the use of muscle biopsies (MB) for the diagnosis of systemic vasculitides (SV). We aimed to evaluate the diagnostic use of MB in this condition. METHODS: We reviewed 310 consecutive MB performed in our center between 2000 and 2008 and correlated them with clinical data from the corresponding patients. Thirty-one of the patients, representing a total of 33 MB, were diagnosed with active SV. MB were considered positive when they demonstrated either necrotizing vasculitis or nonnecrotizing vasculitis. RESULTS: Twenty-two of the 33 MB were positive (sensitivity of 66.7%), with necrotizing vasculitis and nonnecrotizing vasculitis being equally frequent. The SV were antineutrophil cytoplasmic antibody (ANCA)-associated in 22 patients (71%), and ANCA-negative in 9 cases (29%). Neither the type nor the clinical spectrum of the SV was predictive of MB positivity. None of the muscle symptoms (myalgias or biological rhabdomyolysis) were correlated with MB positivity. All the biopsies were performed uneventfully. CONCLUSION: The feasibility and positive predictive value of MB make it a valuable tool for ruling out a diagnosis of SV. Since no clinical signs could predict its positivity, MB should be considered in all suspected cases of SV. Unlike other biopsies, including kidney biopsy, MB had no prognostic value.

[Abdominal aortic involvement in active giant cell (temporal) arteritis: a study of 20 patients by Doppler ultrasonography and computed tomographic angiography]. / Fréquence de l'atteinte de l'aorte abdominale au diagnostic de maladie de Horton : étude de 20 patients par échographie-Doppler et angiotomodensitométrie.

OBJECTIVE: This study used Doppler ultrasonography and computed tomographic angiography (CTA) to assess the prevalence of abdominal aortic lesions that suggested abdominal aortitis at diagnosis of giant-cell or temporal arteritis (GCA). We also evaluated the contribution of these 2 techniques to diagnosis. METHODS: This single-center study included patients admitted to the internal medicine department of the Nantes (France) University Hospital, between May 1999 and May 2002 at the time of their diagnosis with biopsy-proven GCA. In the 8 weeks after diagnosis, patients underwent a thorough workup, including the collection of clinical and laboratory data. Imaging tests for each patient included Doppler ultrasonography and a CTA scan of the abdominal aorta, looking for aneurysms, ectasia, and thickening of the aortic wall. RESULTS: This study included 20 patients (17 women, mean age 73.9+/-7.2 years, mean CRP=116+/-75.9mg/L). Doppler ultrasonography suggested aortitis in 8 cases (40%): 7 patients (35%) had a hypoechoic halo, 3 (15%) a small aneurysm (diameter < 30mm), and 2 (10%) both. CTA scans of the aorta showed aortitis in 5 cases (25%), all with abnormal thickening of the aortic wall. CTA did not identify any aneurysms. Overall, abdominal aortitis was suspected in 10 patients (50%). CONCLUSION: At the time of GCA diagnosis, both Doppler ultrasonography and CTA can detect morphological abnormalities of the abdominal aorta. Here they suggested asymptomatic abdominal aortitis in half the patients. Doppler ultrasonography appears more effective for detecting aortic aneurysms, while CTA seems helpful for the diagnosis of parietal thickening. The risk factors associated with abdominal aortitis in GCA remain to be identified.

Aortic involvement in recent-onset giant cell (temporal) arteritis: a case-control prospective study using helical aortic computed tomodensitometric scan.

OBJECTIVE: The prevalence of the involvement of large vessels in giant cell arteritis (GCA) is 3-13%. Aortitis is the most serious complication of GCA. Computed tomodensitometric (CT) scan allows analysis of both the aortic wall and endoluminal part of the aorta. Therefore, we conducted a study using CT scan to analyze aortic abnormalities in patients with recent-onset GCA. METHODS: This prospective controlled study compared patients with biopsy-proven GCA with a matched control group based on sex, age, and cardiovascular risk factors. During the 4-week period following diagnosis of GCA, patients underwent an aortic CT scan. The aortic imaging results were blindly compared between both groups. RESULTS: From January 5, 1998 to January 11, 1999, 22 patients and 22 controls were screened by CT scan for aortic involvement. Thickening of the aortic wall was more frequent among patients than controls (45.4% versus 13.6%; P = 0.02). Aortic thickening (mean 3.3 mm) was located on the ascending part of the thoracic aorta in 22.7% of the patients, with no evidence of thickening in the controls (P = 0.05). Thickening of the abdominal aortic wall was noted in 27.3% of the patients and none of the controls (P = 0.02). CONCLUSION: This study suggests that inflammatory aortic thickening, detected by CT scan, occurs frequently at the time of diagnosis of GCA, and that this condition predominantly occurs on the ascending part of the aorta.

Vasculitides associated with malignancies: analysis of sixty patients.

OBJECTIVE: To describe characteristics and outcomes of vasculitides associated with malignancies. METHODS: The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy. RESULTS: Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02). CONCLUSION: Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.

Silent versus cranial giant cell arteritis. Initial presentation and outcome of 50 biopsy-proven cases.

BACKGROUND: The objective of the present study was to compare the silent form of giant cell arteritis (GCA) to the classic cephalic form of the disease. METHODS: We conducted a retrospective study based on a chart review of 50 consecutive, biopsy-proven GCA, recorded at a department of internal medicine. We sought to distinguish a silent form, defined by a prolonged inflammatory syndrome or fever of unknown origin with the absence of cephalic signs, polymyalgia rheumatica, or large artery involvement, from an overt "classic" cranial temporal arteritis. RESULTS: The prevalence of the silent form of GCA was 46% in our study. Abnormal temporal arteries were more frequent in the cephalic group. The silent GCA group had higher C-reactive protein levels (p<0.05), a higher platelet count (p<0.05), and lower serum albumin (p<0.05). There was no significant difference in temporal artery specimens in the two groups. Clinical relapses tended to be more frequent, and patients free of corticosteroids tended to be less frequent, in the cephalic group, though the difference was not statistically significant. CONCLUSIONS: The silent and cephalic forms of GCA could have distinct clinical and biological patterns and different outcomes. The limitation of our study was its retrospective design. Further studies are required to determine if this distinction is useful in treating GCA patients.