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"I'm still dad": The Impact of Scleroderma on being a Father.

Actualités En Médecine Interne - il y a 4 heures 19 min

"I'm still dad": The Impact of Scleroderma on being a Father.

Occup Ther Health Care. 2018 Jan;32(1):1-13

Authors: Poole JL, Haygood D, Mendelson C

Abstract
The purpose of this study was to describe the experiences of fathers with scleroderma. Ten fathers with scleroderma were interviewed by telephone. Interviews were tape-recorded and transcribed verbatim. Two key themes emerged related to the emotional impact of the illness and the day to day realities of the illness with the unpredictability and rareness of the illness leading to ongoing feelings of isolation and fear of mortality. The negative influences of being a father with scleroderma included the inability of the fathers to participate in physical activities with their children such as outdoor sports and throwing balls. Being able to spend quality time with the child was a positive influence of the illness.

PMID: 29351518 [PubMed - in process]

[Artificial radial head replacement for the treatment of comminuted fractures of the radial head].

Actualités En Médecine Interne - il y a 4 heures 19 min

[Artificial radial head replacement for the treatment of comminuted fractures of the radial head].

Zhongguo Gu Shang. 2017 Apr 25;30(4):360-363

Authors: Liu LC, Ding WJ, Zhou XX, Zhu DD, Huang JL

Abstract
OBJECTIVE: To assess the clinical effects of artificial radial head prosthesis replacement for the treatment of comminuted fracture of the radial head.
METHODS: From June 2011 to June 2015, 25 patients with radial head comminuted fracture were treated with artificial radial head replacement, including 10 males and 15 females, ranging in age form 24 to 61 years old(mean, 40 years old). The functional recovery of the patients suffering from limb and elbow in different periods, the activity degree of the elbow joint and the function of the elbow in the latest follow-up were compared.
RESULTS: All the patients were followed up, and the duration ranged from 12 to 48 months, averaged 26 months. There were no complications such as infection, elbow instability, subluxation of the distal radioulnar joint, and myositis ossificans. The VAS, Broberg and Morrey elbow function score were improved 6, 9 months after operation compared with that 3 months after operation(P<0.05). There were significant differences in elbow flexion and extension, rotation activity between injured side and healthy side 3, 6, 9 months after operation(P<0.01), but no significant differences between injured side and healthy side at the latest follow-up(P>0.05). At the latest follow-up, according to Broberg and Morrey elbow function evaluation criteria, 16 cases got an excellent result, 7 good and 2 poor.
CONCLUSIONS: It can maximize the recovery of elbow joint stability and quicken early functional exercise, prevent and reduce the occurrence of complications by using the artificial radial head replacement therapy to repair comminuted fracture of the radial head. The short-term curative effect is satisfactory, but the long-term effect needs further observation.

PMID: 29349989 [PubMed - in process]

Matrix Stiffness: the Conductor of Organ Fibrosis.

Actualités En Médecine Interne - il y a 4 heures 19 min

Matrix Stiffness: the Conductor of Organ Fibrosis.

Curr Rheumatol Rep. 2018 Jan 19;20(1):2

Authors: Santos A, Lagares D

Abstract
PURPOSE OF REVIEW: Organ fibrosis is a lethal component of scleroderma. The hallmark of scleroderma fibrosis is extensive extracellular matrix (ECM) deposition by activated myofibroblasts, specialized hyper-contractile cells that promote ECM remodeling and matrix stiffening. The purpose of this review is to discuss novel mechanistic insight into myofibroblast activation in scleroderma.
RECENT FINDINGS: Matrix stiffness, traditionally viewed as an end point of organ fibrosis, is now recognized as a critical regulator of tissue fibrogenesis that hijacks the normal physiologic wound-healing program to promote organ fibrosis. Here, we discuss how matrix stiffness orchestrates fibrosis by controlling three fundamental pro-fibrotic mechanisms: (a) mechanoactivation of myofibroblasts, (b) integrin-mediated latent transforming growth factor beta 1 (TGF-β1) activation, and (c) activation of non-canonical TGF-β1 signaling pathways. We also summarize novel therapeutic targets for anti-fibrotic therapy based on the mechanobiology of scleroderma. Future research on mechanobiology of scleroderma may lead to important clinical applications such as improved diagnosis and treatment of patients with scleroderma and other fibrotic-related diseases.

PMID: 29349703 [PubMed - in process]

Superficial temporal artery aneurysm associated with immunoglobulin G4-related disease.

Actualités En Médecine Interne - il y a 4 heures 19 min

Superficial temporal artery aneurysm associated with immunoglobulin G4-related disease.

J Vasc Surg Cases Innov Tech. 2017 Dec;3(4):243-246

Authors: Kuma S, Takeshima T, Ohga T, Nozoe T, Sueishi K

Abstract
A 68-year-old man was admitted because of a pulsatile mass and pain in the left temporal region, and computed tomography demonstrated the superficial temporal artery aneurysm. He underwent aneurysmectomy, and pathologic investigation revealed marked thickness of the adventitia with substantial plasmacyte infiltration. On immunoglobulin G4 (IgG4) immunohistochemistry, IgG4-positive lymphocytes were scattered in the adventitia, and biochemical tests revealed elevation of IgG4 (200 mg/dL). The case satisfied the criteria for both giant cell arteritis and IgG4-related disease (IgG4-RD). This case report suggested that IgG4-RD can occur in the superficial temporal artery and that IgG4-RD may partially overlap with a subtype of giant cell arteritis.

PMID: 29349436 [PubMed]

Takayasu arteritis with middle aortic syndrome and mesenteric ischemia treated by aortic stenting.

Actualités En Médecine Interne - il y a 4 heures 19 min

Takayasu arteritis with middle aortic syndrome and mesenteric ischemia treated by aortic stenting.

J Vasc Surg Cases Innov Tech. 2017 Sep;3(3):168-170

Authors: Morrissey S, Tan KT, Byrne JS

Abstract
A 48-year-old woman suffering from Takayasu arteritis presented with middle aortic syndrome consisting of abdominal pain, refractory hypertension with pulmonary edema and pleural effusions, and lower limb ischemia. She failed to improve with high-dose steroid therapy and underwent endovascular stenting of two severe stenoses in the supraceliac and infrarenal aorta. Her symptoms resolved and hypertension improved after the procedure.

PMID: 29349411 [PubMed]

Isolated lower extremity vasculitis leading to progressive critical limb ischemia.

Actualités En Médecine Interne - il y a 4 heures 19 min

Isolated lower extremity vasculitis leading to progressive critical limb ischemia.

J Vasc Surg Cases Innov Tech. 2017 Sep;3(3):119-122

Authors: Parsa P, Rios A, Anderson LN, Eidt JF, Gable D, Grimsley B

Abstract
Giant cell arteritis is a large- and medium-vessel vasculitis that has been described as a systemic disease process with disseminated vessel involvement. Advances in vascular imaging techniques have demonstrated that involvement of the large vessels of the upper and lower limbs may be more prevalent than was once thought, although the clinical implications of this are unknown. Isolated lower extremity claudication without systemic or classic cranial symptoms, especially as a primary manifestation of giant cell arteritis, is rare. We present the case of a patient with isolated bilateral limb claudication that rapidly progressed to critical limb ischemia requiring urgent surgical intervention after steroid therapy. Our patient has consented to the publication of this report.

PMID: 29349396 [PubMed]

An immunohistochemical analysis of folate receptor beta expression and distribution in giant cell arteritis - a pilot study.

Actualités En Médecine Interne - il y a 4 heures 19 min

An immunohistochemical analysis of folate receptor beta expression and distribution in giant cell arteritis - a pilot study.

Am J Clin Exp Immunol. 2017;6(6):107-114

Authors: Albano-Aluquin S, Malysz J, Aluquin VR, Ratnam M, Olsen N

Abstract
BACKGROUND: Giant cell arteritis (GCA) is a chronic vasculitis of large and medium vessels in which no targetable biomarkers exist to allow selective treatment, predict disease activity and monitor therapeutic responses. The accessibility of the temporal artery (TA) for biopsy allows morphologic studies to characterize macrophages and T cells in the microenvironment of the arterial wall. We evaluated the expression of folate receptor beta (FRB), a candidate diagnostic/therapeutic biomarker, compared its expression with key macrophage markers and correlated it with GCA severity.
METHODS: Formalin-fixed paraffin-embedded tissue sections were examined from 6 patients with GCA and 2 controls. Immunohistochemistry was performed using FRB, ETB, CD68 and CD3 antibodies to evaluate for activated macrophages and T cells, assess FRB distribution along the intima, media and adventitial layers and composition of inflammatory infiltrates. We compared the expression of FRB, ETB and CD68 in GCA versus negative controls and in severe (with visual loss) versus mild (without visual loss) disease.
RESULTS: In GCA, moderate to severe inflammation was accompanied by >90% destruction of the internal elastic lamina. Macrophages comprised 36.3 ± 4.1% while CD3+ lymphocytes accounted for 61.7 ± 4.1% of total leukocytes. FRB was selectively expressed in macrophages and localized to the adventitia. GCA patients had marginally increased median FRB (9.8 cells/hpf vs. 0; p=0.095), ETB (20.5 vs. 0; p=0.095) and CD68 (38.8 vs. 5; p=0.071) expression versus controls. ETB was found in endothelial cells, smooth muscle cells and macrophages in intima/media. FRB positively correlated with ETB (r=0.90; p-0.037) and CD68 levels (r=0.90; p=0.037). ETB expression positively correlated with CD68 (r=1.0; p<0.0001). There was no difference in FRB between severe and mild GCA.
CONCLUSION: FRB is a potential diagnostic and therapeutic biomarker with restricted expression in GCA macrophages. FRB+ macrophages localized to the adventitia and their expression correlated with ETB and CD68 macrophages, suggesting that they contribute to GCA pathogenesis.

PMID: 29348986 [PubMed]

Granulomatous Pancreas: A Case Report of Pancreatic Sarcoid.

Actualités En Médecine Interne - il y a 4 heures 19 min

Granulomatous Pancreas: A Case Report of Pancreatic Sarcoid.

Case Rep Gastrointest Med. 2017;2017:1620392

Authors: Bihun T, Diaz Y, Wenig S

Abstract
Sarcoidosis is a chronic, systemic, noncaseating granulomatous disease process of unknown etiology. Sarcoidosis most commonly manifests in the lungs; however, gastrointestinal manifestations can occur. If in the GI tract, it is almost always found in the liver. Solitary pancreatic lesions are extremely rare, with less than 50 documented cases found in the literature. We present a case of a 61-year-old female, with a past medical history of sarcoidosis, who presented to the ER with unexpected weight loss, scleral icterus, right upper quadrant pain, and epigastric and back pain. US and MRI found a dilated common bile duct and mild dilation of the pancreatic duct, as well as a focal prominence in the head of the pancreas surrounded by areas of atrophy. A pancreaticoduodenectomy procedure was performed and fresh frozen sections were taken. The pathologist made a diagnosis of nonnecrotizing granulomatous pancreatitis. Pancreatic sarcoid is often asymptomatic and a benign finding on autopsy; however, clinicians should be mindful of pancreatic involvement when working up differential diagnosis for pancreatic masses.

PMID: 29348948 [PubMed]

Disruption of CXCR3 function impedes the development of Sjögren's syndrome-like xerostomia in non-obese diabetic mice.

Actualités En Médecine Interne - il y a 4 heures 19 min

Disruption of CXCR3 function impedes the development of Sjögren's syndrome-like xerostomia in non-obese diabetic mice.

Lab Invest. 2018 Jan 18;:

Authors: Zhou J, Yu Q

Abstract
The chemokine receptor CXCR3 plays an important role in T cell recruitment in various immune responses and autoimmune diseases. Expression of CXCR3 ligands, including CXCL9, CXCL10, and CXCL11, is elevated in the salivary glands of patients with Sjögren's syndrome (SS). To elucidate whether interaction between CXCR3 and its ligands is required for the development of SS, we administrated an anti-CXCR3 blocking antibody (CXCR3-173) to the non-obese diabetic (NOD) mice, a well-defined model of SS, during the stage prior to disease onset. Treatment with this anti-CXCR3 antibody significantly improved salivary secretion, indicating a remission of SS clinical manifestation. Anti-CXCR3 treatment did not affect the gross leukocyte infiltration of the submandibular glands (SMGs) as assessed by hematoxylin and eosin staining. However, flow cytometric analysis showed that anti-CXCR3 treatment markedly reduced the percentage of CXCR3+CD8 T and CXCR3+CD44+CD8 T cells, without affecting that of CXCR3+CD4 T and CXCR3+CD44+CD4 T cells in the SMGs and submandibular lymph nodes, suggesting a preferential effect of this anti-CXCR3 treatment on CXCR3-expressing effector CD8 T cells. Meanwhile, SMG expression of inflammatory factor TNF-α was markedly diminished by anti-CXCR3 treatment. In accordance, anti-CXCR3 significantly enhanced SMG expression of tight junction protein claudin-1 and water channel protein aquaporin 5, two molecules that are crucial for normal salivary secretion and can be down-regulated by TNF-α. Taken together, these findings demonstrated that the interaction between the endogenous CXCR3 and its ligands plays a pro-inflammatory and pathogenic role in the development of SS-like xerostomia in the NOD mouse model.

PMID: 29348563 [PubMed - as supplied by publisher]

Daily home spirometry to detect early steroid treatment effects in newly treated pulmonary sarcoidosis.

Actualités En Médecine Interne - il y a 4 heures 19 min

Daily home spirometry to detect early steroid treatment effects in newly treated pulmonary sarcoidosis.

Eur Respir J. 2018 Jan;51(1):

Authors: Broos CE, Wapenaar M, Looman CWN, In 't Veen JCCM, van den Toorn LM, Overbeek MJ, Grootenboers MJJH, Heller R, Mostard RL, Poell LHC, Hoogsteden HC, Kool M, Wijsenbeek MS, van den Blink B

PMID: 29348185 [PubMed - in process]

Temporal artery biopsy: time matters!

Actualités En Médecine Interne - il y a 4 heures 19 min
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Temporal artery biopsy: time matters!

Intern Med J. 2017 12;47(12):1465

Authors: Sait MR, Lepore M, Kwasnicki R, Allington J, Srinivasaiah N

PMID: 29224203 [PubMed - indexed for MEDLINE]

Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients.

Actualités sur la maladie lupique - il y a 11 heures 2 min
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Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients.

Medicine (Baltimore). 2017 Dec;96(48):e9017

Authors: Touzot M, Terrier CS, Faguer S, Masson I, François H, Couzi L, Hummel A, Quellard N, Touchard G, Jourde-Chiche N, Goujon JM, Daugas E, Groupe Coopératif sur le Lupus Rénal (GCLR)

Abstract
Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy. Electron microscopy was retrospectively performed when the material was available. End points were the proportion of patients with a complete response (urine protein to creatinine ratio <0.5 g/day and a normal or near-normal eGFR), partial response (≥50% reduction in proteinuria to subnephrotic levels and a normal or near-normal eGFR), or nonresponse at 12 months or later after the initiation of the treatment.The study included 12 patients (66% female) with a median age of 36.5 years. At diagnosis, median creatinine and proteinuria levels were 1.21 mg/dL (range 0.5-11.6) and 7.5 g/day (1.4-26.7), respectively. Six patients had nephrotic syndrome and acute kidney injury. Renal biopsy examinations revealed class III or class IV A/C lupus nephritis in all cases. Electron microscopy was performed on samples from 5 patients. The results showed mesangial and subendothelial dense deposits consistent with LN in 4 cases, and a retrospective diagnosis of pseudo-amyloid fibrillary glomerulonephritis was made in 1 patient.Patients received immunosuppressive therapy consisting of induction therapy followed by maintenance therapy, similar to treatment for severe lupus nephritis. Remission was recorded in 10 patients at 12 months after the initiation of treatment. One patient reached end-stage renal disease. After a median follow-up of 24 months, 2 patients relapsed.Lupus nephritis in the absence of overt SLE is a nosological entity requiring careful etiological investigation, including systematic electron microscopy examination of renal biopsies to rule out fibrillary glomerulonephritis. In this series, most patients presented with severe glomerulonephritis, which was highly similar to lupus nephritis at presentation and in terms of response to immunosuppressive therapy.

PMID: 29310419 [PubMed - indexed for MEDLINE]

Catégories: Lupus systémique

Secondary systemic lupus erythematosus after thymoma resection misdiagnosed as medically unexplained dyspnoea: A case report.

Actualités sur la maladie lupique - il y a 11 heures 2 min
Related Articles

Secondary systemic lupus erythematosus after thymoma resection misdiagnosed as medically unexplained dyspnoea: A case report.

Medicine (Baltimore). 2017 Dec;96(48):e8944

Authors: Liao H, Pei W, Liu J, Wang K, Xu T, Chen X

Abstract
RATIONALE: Secondary systemic lupus erythematosus (SLE) is an exceedingly rare complication of thymoma resection and is difficult to diagnose because of the insidious and nonspecific clinical manifestations. A case of SLE that occurs secondary to thymoma resection is described in this report.
PATIENT CONCERNS: A 43-year-old male came to our hospital with the sole symptom of dyspnea after thymoma resection initially. However, other atypical lesions of SLE occurred over time.
DIAGNOSES: Antinuclear antibody spectrum test showed positive results and the diagnosis of SLE was obtained.
INTERVENTIONS: Initially the patient was treated for medically unexplained dyspnea (MUD) without much improvement. Following the diagnosis, the methylprednisolone pulse therapy and therapies of immunoglobulin and cyclophosphamide were adopted for the treatment.
OUTCOMES: Finally, the patient's symptoms faded rapidly and favorable prognosis has been maintained till now.
LESSONS: This case highlights the importance of a serious and comprehensive analysis before we give the diagnosis of MUD. Additionally, ignorance of secondary SLE after thymoma resection should be prevented to avoid a delayed diagnosis and treatment.

PMID: 29310389 [PubMed - indexed for MEDLINE]

Catégories: Lupus systémique

Scleroderma renal crisis and cancer.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55

Scleroderma renal crisis and cancer.

QJM. 2018 Jan 15;:

Authors: Crosnier C, Achille A, Lecouffe-Desprets M, Néel A, Durant C, Agard C

Abstract
We report two cases of elderly patients with diffuse systemic sclerosis (SSc) and inaugural scleroderma renal crisis (SRC). Initial exhaustive search for an underlying cancer was negative, but aggressive metastatic neoplasia was diagnosed after 1 year of follow-up. We emphasize the role of 18-FDG PET-scan to detect neoplasia in the following months after inaugural SRC in elderly.

PMID: 29346637 [PubMed - as supplied by publisher]

Sarcoidosis With Pleural Effusion as the Presenting Symptom.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55

Sarcoidosis With Pleural Effusion as the Presenting Symptom.

J Bronchology Interv Pulmonol. 2018 Jan 16;:

Authors: Rivera E, Gesthalter Y, Vardelaan P, Chee A, Majid A

Abstract
A 65-year-old woman, never smoker, with medical history of hypertension, nonischemic cardiomyopathy, and moderate pulmonary hypertension presented with symptomatic bilateral pleural effusions. Thoracentesis revealed a lymphocyte predominant transudate and was negative for malignancy, microbiologic cultures were negative for an infectious cause. Chest tomography showed mediastinal and bilateral hilar lymphadenopathy, lymph node biopsy with endobronchial ultrasound-guided transbronchial needle aspiration showed non-necrotizing granulomas compatible with sarcoidosis. Echocardiogram showed ejection fraction of 45% and cardiac workup for sarcoid involvement was negative. Despite overall clinical management with diuretics, pleural effusion persisted and the patient underwent medical thoracoscopy with pleural biopsy. Biopsy showed noncaseating granulomas consistent with sarcoid, with all stains and microbiologic cultures negative for an infectious etiology. To the best of our knowledge, this is the first described case of sarcoidosis presenting as large transudative pleural effusion.

PMID: 29346252 [PubMed - as supplied by publisher]

Incidence, Etiology, and Clinicopathologic Features of Endobronchial Benign Lesions: A 10-Year Consecutive Retrospective Study.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55

Incidence, Etiology, and Clinicopathologic Features of Endobronchial Benign Lesions: A 10-Year Consecutive Retrospective Study.

J Bronchology Interv Pulmonol. 2018 Jan 16;:

Authors: Marchioni A, Casalini E, Andreani A, Cappiello G, Castaniere I, Fantini R, Cecilia Mengoli M, Tonelli R, Clini EM, Rossi G

Abstract
BACKGROUND: Airways can be affected by non-neoplastic lesions leading to critical stenosis of the lumen. Incidence, etiology, and clinical significance of endobronchial benign lesions are not systematically characterized.This study aimed to assess the epidemiology of nonmalignant processes involving the bronchial tree on clinical, pathologic, endoscopic, and radiologic grounds.
METHODS: We retrospectively analyzed bronchoscopy procedures over 10 years at the Bronchoscopy Unit of Modena University Hospital. All the endoscopically growing benign lesions with histologic confirmation were considered. For each lesion, we evaluated demographics, clinical features and outcome, the endoscopic aspect and radiologic characteristics by means of computed tomography as assessed by 2 experienced radiologists blinded with regard to the diagnosis.
RESULTS: Over the study period, we analyzed 10,431 bronchoscopies and identified 2075 cases of tracheobronchial alterations. Among these, 11.2% had a benign etiology with an average annual incidence of 23 new cases/year and a general incidence of 2.2%. Anthrachosis was the most prevalent bronchial lesion. In total, 22% of benign lesions presented airway stenosis >50% and required bronchoscopic treatment. Bronchial stenosis was most frequently observed in tuberculosis (P=0.031) and aspergillosis (P=0.020) when compared with sarcoidosis. Immunosuppressive status was significantly associated with endobronchial aspergillosis (P=0.0001) and the 1-year survival from diagnosis resulted significantly lower irrespectively to the immune system condition.
CONCLUSIONS: A consistent proportion of endobronchial benign lesions are reported. One fifth of these are associated with critical stenosis of the airway lumen, requiring rigid bronchoscopy. Among these, aspergillosis is characterized by the poorest prognosis, regardless of host immunity status.

PMID: 29346250 [PubMed - as supplied by publisher]

Preeclampsia and scleroderma: a prospective nation-wide analysis.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55
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Preeclampsia and scleroderma: a prospective nation-wide analysis.

Acta Obstet Gynecol Scand. 2018 Jan 18;:

Authors: Kamper-Jørgensen M, Gammill HS, Nelson JL

Abstract
In a preliminary case-control study, women with scleroderma more frequently reported having had hypertensive complications during pregnancy compared with healthy women. To prospectively investigate this possible association, we conducted a nation-wide cohort analysis of a major hypertensive complication during pregnancy, namely preeclampsia, and later scleroderma. Analyses were based on Danish register-based birth and hospital contact data on preeclampsia and scleroderma. We followed 778,758 women from time of giving birth between 1978 and 2010 to end of follow-up, emigration, death, or scleroderma diagnosis, whichever occurred first. The association was evaluated by incidence rate ratios, obtained in Poisson regression models. We report that preeclampsia is associated with a 69% significantly increased risk of later developing scleroderma. Though these findings do not impact clinical care directly, the association of preeclampsia with scleroderma underscores the significant relationship of preeclampsia and other adverse pregnancy outcomes with later disease in women and should be included in patient counseling and education. This article is protected by copyright. All rights reserved.

PMID: 29344946 [PubMed - as supplied by publisher]

Giant Cell Arteritis: Practical Pearls and Updates.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55
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Giant Cell Arteritis: Practical Pearls and Updates.

Curr Pain Headache Rep. 2018 Jan 17;22(1):2

Authors: Pradeep S, Smith JH

Abstract
PURPOSE OF REVIEW: The purpose of this review is to summarize recent updates and distill practical points from the literature which can be applied to the care of patients with suspected and confirmed giant cell arteritis (GCA).
RECENT FINDINGS: Contemporary thinking implicates a fundamental failure of T regulatory cell function in GCA pathophysiology, representing opportunity for novel therapeutic avenues. Tocilizumab has become the first Food and Drug Administration-approved treatment for GCA following demonstration of efficacy and safety in a phase 3 clinical trial. There have been significant parallel advances in both our understanding of GCA pathophysiology and treatment. Tocilizumab, and other agents currently under investigation in phase 2 and 3 clinical trials, presents a new horizon of hope for both disease remission and avoidance of glucocorticoid-related complications.

PMID: 29344777 [PubMed - in process]

Peptide receptor radionuclide therapy as a new tool in treatment-refractory sarcoidosis - initial experience in two patients.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55
Related Articles

Peptide receptor radionuclide therapy as a new tool in treatment-refractory sarcoidosis - initial experience in two patients.

Theranostics. 2018;8(3):644-649

Authors: Lapa C, Kircher M, Hänscheid H, Schirbel A, Grigoleit GU, Klinker E, Böck M, Samnick S, Pelzer T, Buck AK

Abstract
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that can involve virtually all organ systems. Whereas most patients present without symptoms, progressive and disabling organ failure can occur in up to 10% of subjects. Somatostatin receptor (SSTR)-directed peptide receptor radionuclide therapy (PRRT) has recently received market authorization for treatment of SSTR-positive neuroendocrine tumors. Methods: We describe the first case series comprising two patients with refractory multi-organ involvement of sarcoidosis who received 4 cycles of PRRT. Results: PRRT was well-tolerated without any acute adverse effects. No relevant toxicities could be recorded during follow-up. Therapy resulted in partial response accompanied by a pronounced reduction in pain (patient #1) and stable disease regarding morphology as well as disease activity (patient #2), respectively. Conclusion: Peptide receptor radionuclide therapy in sarcoidosis is feasible and might be a new valuable tool in patients with otherwise treatment-refractory disease. Given the long experience with and good tolerability of PRRT, further evaluation of this new treatment option for otherwise treatment-refractory sarcoidosis in larger patient cohorts is warranted.

PMID: 29344295 [PubMed - in process]

Coexistence of sarcoidosis and metastatic lesions: A diagnostic and therapeutic dilemma.

Actualités En Médecine Interne - ven, 19/01/2018 - 14:55
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Coexistence of sarcoidosis and metastatic lesions: A diagnostic and therapeutic dilemma.

Oncol Lett. 2017 Dec;14(6):7643-7652

Authors: Spiekermann C, Kuhlencord M, Huss S, Rudack C, Weiss D

Abstract
Sarcoidosis, a chronic, inflammatory disease that affects various different organs, is characterized by noncaseating epitheloid granulomas. This systemic inflammatory process is associated with an increased risk of cancer. Several cases of sarcoidosis that mimic metastatic tumor progression in radiological findings have been reported so far. However, there are also cases that have presented a coexistence of sarcoidosis and metastasis, which have caused a diagnostic and therapeutic dilemma. Due to inadequate current therapies, a reliable differentiation between benign and malignant lesions is crucial. This review focuses on the residual risk of the coexistence of metastases within radiological suspicious lesions in patients with a history of solid tumors and sarcoidosis, as well as immunological findings, in order to explain the potential associations. Sarcoidosis has the potential to promote metastasis as it includes tumor-promoting and immune-regulating cell subsets. Notably, myeloid derived suppressor cells may serve a pivotal role in metastatic progression in patients with sarcoidosis. In addition, the present review also evaluates the potential novel diagnostic approaches, which may be able to differentiate between metastatic lesions and sarcoidosis. The risk of coexistent metastasis in sarcoidosis lesions must be considered by clinical practitioners, and a multidisciplinary approach may be required to avoid misdiagnosis and the subsequent unnecessary surgery or insufficient treatments.

PMID: 29344212 [PubMed]

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