Myocardial tissue characterization in systemic lupus erythematosus: value of a comprehensive cardiovascular magnetic resonance approach.

Systemic lupus erythematosus (SLE) is a multi-organ inflammatory disorder mainly affecting women and is associated with high cardiovascular morbidity and mortality. We tested the utility of a comprehensive cardiovascular magnetic resonance approach to assess myocardial involvement and to determine its relation to disease activity in SLE patients. We studied 20 SLE patients (19 females, 35+/-10 years) and 13 healthy volunteers (nine females, 28+/-11 years). Classification followed the criteria of the American College of Rheumatology and assessment of SLE activity was based on the European Consensus Lupus Activity Measurement index. Cardiovascular magnetic resonance (CMR) was performed on a 1.5T scanner and included the following sequences: steady-state free precession, T2-weighted, early and late T1-weighted after gadolinium-DTPA injection. Ejection fraction was not significantly different between groups (controls: 63+/-6, inactive SLE: 67+/-7, active SLE 64+/-8; P=0.003 for all groups). In contrast, relative T2 ratio (myocardium to skeletal muscle) was significantly higher in active SLE than in the other groups (controls: 1.7+/-0.3, inactive: 1.8+/-0.2, active: 2.1+/-0.2; P=0.003). Similarly, early enhancement ratio was significantly higher in active SLE (controls: 2.4+/-1.4, inactive: 2.8+/-1.1, active: 4.5+/-2.0, P=0.39). Both relative T2 and early enhancement ratios significantly correlated with disease activity. Intramural foci of late enhancement were observed in three of eight patients (all with active SLE). Of the five patients with no late enhancement, only one had active disease. An imaging approach combining T2-weighted, early and late enhancement imaging is a useful tool to assess possible myocardial involvement in SLE. CMR parameters of global myocardial involvement correlate well with disease activity, but not with usual clinical signs as summarized in a cardiac score.

Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis.

OBJECTIVE: To describe characteristic ultrasound findings and clinical features of patients with newly diagnosed cranial and large-vessel (LV) GCA in a specialized ultrasound clinic. METHODS: This case-control study includes all consecutive patients between 1997 and 2006 with newly diagnosed GCA. Duplex ultrasound of the temporal, subclavian, axillary and proximal brachial arteries was performed in all patients with suspected temporal arteritis, PMR, arm claudication, unclear inflammation or pyrexia of unknown origin (PUO). RESULTS: In 53 of 176 patients, ultrasound depicted characteristic vasculitic homogeneous wall swelling of the axillary, subclavian and/or proximal brachial arteries. These were affected in 98, 61 and 21%, respectively, in the 53 patients. The findings were bilateral in 79%. Axillary arteries were stenotic or occluded in 51 and 2% and temporal artery ultrasound and histology were positive in 62 and 67% of LV-GCA cases, respectively. A significantly greater number of LV-GCA patients were female (83 vs 65%) and younger (mean 66 vs 72 yrs) as compared with those without proximal arm involvement. Headaches (38 vs 75%), jaw claudication (24 vs 48%) and anterior ischaemic optic neuropathy (4 vs 19%) occurred significantly less frequently. The median time until diagnosis was significantly longer (31 vs 8 weeks). ESR and presence of PMR were similar in both groups. CONCLUSIONS: Performing axillary artery ultrasound in all patients with suspected temporal arteritis, PMR, arm claudication, unclear inflammation or PUO increases the diagnostic yield for LV-GCA. Patients with LV-GCA differ from those without arm involvement.

[Thrombotic thrombocytopenic purpura - a rare cause of thrombocytopenia in systemic lupus erythematosus]. / Thrombotisch-thrombozytopenische Purpura - seltene Ursache einer Thrombozytopenie bei systemischem Lupus erythematodes.

HISTORY AND ADMISSION FINDINGS: An 18-year old woman with a systemic lupus erythematosus was referred to our hospital with anaemia, thrombocytopenia and neurological symptoms. Skin haemorrhages had occurred some weeks before. The patient also noticed cardiac palpitations. After the primary care doctor had increased the dosage of prednisolone from 7.5 mg per day to 20 mg per day the symptoms didn't substantially change and that the patient was referred to hospital. An acute lupus flare was assumed. INVESTIGATIONS: Laboratory examinations showed a Coombs-negative haemolytic anaemia with schistocytes. Von Willebrand factor(vWF)-cleaving protease was decreased. The presence of an inhibitor of the enzyme was proved. Further test results as anti-ds-DNA, anti-Sm, C 3, ESR, CRP were normal. C 4 was slightly decreased. Cerebral MRI, cerebral SPECT and Duplex sonography of the extracranial arteries didn't show any pathologic findings. The performed MRI of the heart revealed signs of myocardial damage. DIAGNOSIS, TREATMENT AND COURSE: As cause of the recent symptoms a thrombotic-thrombocytopenic purpura (TTP) was diagnosed. This obviously was due to an auto-antibody against the vWF-cleaving protease. After intensive immunosuppressive therapy with prednisolone and cyclosporin the clinical and laboratory findings normalised. An acute therapy with fresh frozen plasma and plasmapheresis was not necessary, as the symptoms were moderate. Because of the transient cerebral deficits for a short time we administered a low dose of aspirin. The MRI signs of myocardial affection didn't change after 3 months of immunosuppressive treatment so that we started an ACE inhibitor treatment. CONCLUSION: Thrombocytopenia in systemic lupus erythematosus demands an individual differential diagnosis. Rare causes as TTP have to be excluded. This disorder has to be diagnosed quickly and the adequate therapy has to be started early.

Involvement of peripheral arteries in giant cell arteritis: a color Doppler sonography study.

OBJECTIVE: To investigate the involvement of arteries other than the temporal arteries in active giant cell arteritis using color Doppler sonography. METHODS: The occipital, facial, vertebral, carotid, subclavian, axillary, brachial, ulnar radial, femoral, popliteal, posterior tibial, and dorsal pedal arteries, and the abdominal aorta of 33 consecutive patients with acute giant cell arteritis and 33 age- and sex-matched controls were investigated. RESULTS: In 10 patients (30%), but in none of the controls, a characteristic inflammatory mural thickening (halo) could be demonstrated in these arteries. The subclavian, external carotid, and/or facial arteries were involved in 4 patients, the occipital and/or axillary arteries in 3 patients, the brachial and/or ulnar arteries in 2 patients, and the common carotid, vertebral, popliteal, and/or radial arteries in 1 patient each. Two patients had symptomatic large vessel giant cell arteritis with arm claudication. The other patients were asymptomatic concerning the involved arteries. Furthermore the ulnar artery was occluded in 3 cases, the posterior tibial artery in 2 cases, and the dorsal pedal and the vertebral artery in 1 case each. No occlusions were found in the controls. Occlusion of the temporal arteries occurred more frequently in patients with peripheral artery involvement than in those without peripheral involvement (60% versus 26%). In most of the non-stenotic, small arteries the halo disappeared within 9 to 21 days. Mural thickening remained in large, stenotic arteries. CONCLUSION: Peripheral artery involvement occurs more frequently in acute temporal arteritis than has been assumed up to now. Color Doppler sonography offers a new method to evaluate this peripheral involvement.

[Hypertrophic osteoarthropathy and thrombocytopenia in bronchial carcinoma]. / Hypertrophische Osteoarthropathie und Thrombozytopenie bei Bronchialkarzinom.

SYMPTOMS: A 40-year-old woman presented with an arthritis of the knees and ankles, digital clubbing and thrombocytopenia. EXAMINATIONS: Blood tests proved an inflammatory reaction. Clinical findings were the above mentioned oligoarthritis and a periostitis of the long extremity bones. Chest X-ray showed a lung tumor. THERAPY: After surgical treatment of the lung cancer both arthritis and thrombocytopenia normalized. CONCLUSION: Hypertrophic osteoarthropathy is characterized by the combination of clubbing, arthropathy and periostitis. This paraneoplasia may be the presenting manifestation of occult malignancy. The diagnosis of hypertrophic osteoarthropathy should cause an intensive search of cancer. Further associated diseases are discussed.