scholarly journals The management of large vessel vasculitides

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Elena Galli ◽  
Chiara Marvisi ◽  
Gilda Sandri ◽  
Carlo Umberto Manzini ◽  
Carlo Salvarani

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) represent the most common large vessel vasculitides (LVV). An early recognition of these conditions is crucial in order to start a prompt treatment to prevent severe ischemic complications, such as irreversible visual loss in GCA and cardiovascular or cerebrovascular accidents in TAK. Isolated glucocorticoids (GCs) still remain the cornerstone of GCA therapy. However, long-term treatment with GCs is burdened by an important toxicity. Furthermore, relapses are frequent during the follow-up period and relapsing patients have to cope with a longer duration of the GC therapy and a higher cumulative GC dose. On the other hand, TAK treatment usually relies on immunosuppressors in addition to GCs from the beginning. Also, since TAK patients are in general young women with a progressive disease, it is essential to treat this vasculitis with steroidsparing drugs in order to avoid excessive GC exposure. For this reason, efforts have been made to discover new therapeutic options able to reduce the cumulative GC dose that is strictly related to GC-toxicity. In recent years, new advances in the management of LVV have become available and have changed the therapeutic approach to these diseases. The aim of this review is to report new evidence of treatment efficacy and safety in LVV.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Dioni Garate ◽  
Adriana Villarreal ◽  
Erika Chiu ◽  
Elizabeth Salsavilca

Abstract Background. Pseudohypoparathyroidism is a heterogeneous condition characterized by hypocalcaemia and hyperphosphatemia as a result of the resistance of the target organ to parathyroid hormone (PTH) 1. It is classified into several different entities (1A, 1B, 1C AND 2) according to molecular and clinical causes. PHP1B has resistance to PTH, normal levels of Gsα activity and has no clear signs of hereditary Albright osteodystrophy (AHO) or other hormonal resistance. 2 Clinical case. A 26-year-old woman was admitted to the emergency service due to an abortion and severe hypocalcemia. Background: generalized tonic-clonic seizures and hypocalcemia for nine years without treatment. The physical exam showed Chvostek and Trousseau, and round face. Its height is 148 cm, weight 60 kg, BMI of 27 kg/m2, without clinical features of AHO. Analytics reported Calcium 4.6 mg / dl (8.5-10.5), βHCG 1795 mIU / ml (<10), PTH 206 pg / ml (15-65), phosphorus 7.35 mg / dl (2.5-4.9) Creatinine 0.57 mg / dl, magnesium 2.02 mg / dl, 25-OH-D3 29ng/ml (>30), TSH 4.2uUI /ml (0.27-4.20), Hb 10.6 mg/dl. A brain CT scan showed calcifications in the basal ganglia (thick annular), subcortical area (crescent) and cerebellar hemispheres. Renal and thyroid ultrasound without alterations. Radiographs of four limbs and skull found no radiological signs of AHO. Ophthalmologic evaluation revealed bilateral cataract.During hospitalization she underwent curettage, was treated with calcium gluconate, then calcium and calcitriol supplements. At follow-up, serum and urine calcium levels were monitored for optimal treatment. Conclusions. We report a patient with typical biochemical findings of PHP and in the absence of AHO it would be classified as 1B. The importance of early recognition and a mandatory multidisciplinary approach offer a better prognosis avoiding extensive brain calcifications, seizures and obstetric complications. The long-term treatment of hypocalcemia associated with resistance to PTH is similar but generally more aggressive than that of primary hypoparathyroidism. 1. Linglart A, Levine M, Juppner H. Pseudohypoparathyroidism. Endocrinol Metab Clin North Am 2018; 47: 865-888. 2. Mantovani G, Bastepe M, et al. Diagnosis and treatment of pseudohypoparathyroidism and related disorders: first declaration of international consensus. Nat Rev Endocrinol. 2018; 14 (8): 476-500.


2013 ◽  
Author(s):  
Christina Marel ◽  
Maree Teesson ◽  
Shane Darke ◽  
Katherine Mills ◽  
Joanne Ross ◽  
...  

2020 ◽  
Author(s):  
Na Wu ◽  
Yuhong Chen ◽  
Yaping Yang ◽  
Xinghuai Sun

Abstract Background: To investigate the corneal biomechanical changes in primary open angle glaucoma (POAG) patients treated with long-term prostaglandin analogue (PGA). Methods: 111 newly diagnosed POAG patients, including 43 high tension glaucoma (HTG) and 68 normal tension glaucoma (NTG), were measured by Corvis ST to obtain intraocular pressure (IOP), central corneal thickness (CCT) and corneal biomechanical parameters at baseline and at each follow-up visit after initiation of PGA treatment. The follow-up measurements were analyzed by the generalized estimate equation model with an exchangeable correlation structure. Restricted cubic spline was employed to estimate the dose-response relation between follow-up time and corneal biomechanics.Results: The mean follow-up time was 10.3 ± 7.02 months. Deformation amplitude (β=-0.0015, P=0.016), the first applanation velocity (AV1, β=-0.0004, P=0.00058) decreased and the first applanation time (AT1, β=0.0089, P<0.000001) increased statistically significantly with PGA therapy over time after adjusting for age, gender, axial length, corneal curvature, IOP and CCT. In addition, AT1 was lower (7.2950 ± 0.2707 in NTG and 7.5889 ± 0.2873 in HTG, P=0.00011) and AV1 was greater (0.1478 ± 0.0187 in NTG and 0.1314 ± 0.0191 in HTG, P=0.00002) in NTG than in HTG after adjusting for confounding factors.Conclusions: Chronic use of PGA probably influences the corneal biomechanical properties directly, which is to make cornea less deformable. Besides, corneas in NTG tended to be more deformable compared to those in HTG with long-term treatment of PGA.


Author(s):  
Gennaro Ratti ◽  
Antonio Maglione ◽  
Emilia Biglietto ◽  
Cinzia Monda ◽  
Ciro Elettrico ◽  
...  

Long term treatment with ticagrelor 60 mg and low-dose aspirin are indicated after acute coronary syndrome (ACS). We retrospectively reviewed aggregate data of 187 patients (155 M and 38 F) (mean age 63.8±9 years) in follow up after ACS with at least one high risk condition (Multivessel disease, diabetes, GFR<60 mL/min, history of prior myocardial infarction, age >65 years) treated with ticagrelor 60 mg twice daily (after 90 mg twice daily for 12 months). The results were compared with findings (characteristics of the patients at baseline, outcomes, bleeding) of PEGASUS-TIMI 54 trial and Eu Label. The highrisk groups were represented as follows: multivessel disease 105 pts (82%), diabetes 63 pts (33%), GFR< 60 mL/min 27 pts (14%), history of prior MI 33 pts (17%), >65 year aged 85 pts (45%). Treatment was withdrawn in 7 patients: 3 cases showed atrial fibrillation and were placed on oral anticoagulant drugs, one developed intracranial bleeding, in three patients a temporary withdrawal was due to surgery (1 colon polyposis and 2 cases of bladder papilloma). Chest pain without myocardial infarction occurred in 16 patients (revascularization was required in 9 patients). Dyspnea was present in 15 patients, but was not a cause for discontinuation of therapy. Long term treatment with ticagrelor 60 mg twice daily plus aspirin 100 mg/day showed a favourable benefit/risk profile after ACS.  In this study all patients had been given ticagrelor 90 mg twice daily for 12 months and the 60 mg twice daily dosage was started immediately thereafter, unlike PEGASUS-TIMI 54 trial in which it was prescribed within a period ranging from 1 day to 1 year after discontinuation of the 90 mg dose. This makes our results more consistent with current clinical practice. However, a careful outpatient follow-up and constant counseling are mandatory to check out compliance to therapy and adverse side effects.


1998 ◽  
Vol 26 ◽  
pp. 103-110 ◽  
Author(s):  
D. Cucinotta ◽  
D. De Leo ◽  
L. Frattola ◽  
M. Trabucchi ◽  
M.G. Albizatti ◽  
...  

1983 ◽  
pp. 511-516
Author(s):  
J.R. JUTTMANN ◽  
D.H. BIRKENHÄGER-FRENKEL ◽  
T.J. VISSER ◽  
C. VAN KRIMPEN ◽  
J.C. BIRKENHÄGER

2020 ◽  
Vol 22 (12) ◽  
Author(s):  
Andriko Palmowski ◽  
Frank Buttgereit

Abstract Purpose While glucocorticoids (GCs) are effective in large vessel vasculitis (LVV), they may cause serious adverse events (AEs), especially if taken for longer durations and at higher doses. Unfortunately, patients suffering from LVV often need long-term treatment with GCs; therefore, toxicity needs to be expected and countered. Recent Findings GCs remain the mainstay of therapy for both giant cell arteritis and Takayasu arteritis. In order to minimize their toxicity, the following strategies should be considered: GC tapering, administration of conventional synthetic (e.g., methotrexate) or biologic (e.g., tocilizumab) GC-sparing agents, as well as monitoring, prophylaxis, and treatment of GC-related AEs. Several drugs are currently under investigation to expand the armamentarium for the treatment of LVV. Summary GC treatment in LVV is effective but associated with toxicity. Strategies to minimize this toxicity should be applied when treating patients suffering from LVV.


2019 ◽  
Vol 44 (1) ◽  
pp. 67-71 ◽  
Author(s):  
Alessandra Voltolini ◽  
Gerardo Salvato ◽  
Maria Frigerio ◽  
Manlio Cipriani ◽  
Enrico Perna ◽  
...  

2020 ◽  
Vol 9 (7) ◽  
pp. 2097 ◽  
Author(s):  
Simon Chauveau ◽  
Florence Jeny ◽  
Marie-Emeline Montagne ◽  
Rola Abou Taam ◽  
Véronique Houdouin ◽  
...  

(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (≤15-year-old) who completed at least a three-year follow-up in French expert centers were included. Clinical information at presentation and outcome in adulthood were studied. (3) Results: A total of 52 patients were included (34 prospectively in childhood and 18 retrospectively in adulthood), with a mean age of 12 (±2.7) at diagnosis. The median duration time of follow-up was 11.5 years (range 3–44.5). Relapses mostly occurred during treatment decrease (84.5%), others within the three years after treatment interruption (9.1%), and rarely when the disease was stable for more than three years (6.4%). Sarcoidosis was severe in 11 (21.2%) in adulthood. Patients received a high corticosteroid cumulative dose (median 17,900 mg) for a median duration of five years (range 0–32), resulting in mostly mild (18; 35.3%) and rarely severe (2; 3.8%) adverse events. (4) Conclusions: Pediatric-onset sarcoidosis needed a long-term treatment in almost half of the patients. Around one fifth of pediatric-onset sarcoidosis patients had severe sarcoidosis consequences in adulthood.


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