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Acute myocardial infarction as the first manifestation of Takayasu arteritis: A case report.

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Acute myocardial infarction as the first manifestation of Takayasu arteritis: A case report.

Medicine (Baltimore). 2019 Apr;98(15):e15143

Authors: Zhang T, Peng B, Tu X, Zhang S, Zhong S, Cao W

Abstract
RATIONALE: Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its major branches. Initial diagnosis is usually difficult due to the highly variable symptoms. Acute myocardial infarction (AMI) is a very rare presentation in patients with TA. Moreover, the choice of early management for these patients is not well established.
PATIENT CONCERNS: A 34-year-old woman was taken to the Emergency Department of our hospital, presenting with a sudden onset and persistent retrosternal chest pain radiating to both upper extremities for 2 hours. Blood pressures were different between 2 arms with 151/115 mm Hg on the right arm and 140/100 mm Hg on the left arm.
DIAGNOSES: The patient was diagnosed with TA according to the medical history, physical examination, and vascular imaging.
INTERVENTIONS: Primary percutaneous coronary intervention (PPCI) was performed to restore the coronary flow of left anterior descending. Meanwhile, combination of oral glucocorticoids and immunosuppressive agents was administered to halt disease progression of TA.
OUTCOMES: Chest pain was relieved without rest and exertional angina. The patient achieved long-term remission without symptom relapse during our follow-up.
LESSONS: Percutaneous coronary intervention was essential and effective in AMI of TA. Timely immunosuppressive therapy could improve the long-term outcome.

PMID: 30985685 [PubMed - indexed for MEDLINE]

Anaphylactoid Syndrome of Pregnancy.

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Anaphylactoid Syndrome of Pregnancy.

Nurs Womens Health. 2019 Feb;23(1):38-48

Authors: Barnhart ML, Rosenbaum K

Abstract
Anaphylactoid syndrome of pregnancy (ASP) is a widespread, proinflammatory, anaphylactic-like reaction that can occur when amniotic fluid enters the maternal blood circulation. ASP is characterized by four cardinal findings: respiratory distress, altered mental status, hypotension, and disseminated intravascular coagulation. ASP is commonly associated with maternal and neonatal mortality. Early recognition followed by prompt and aggressive treatment can improve survival rates and are among the most critical activities for nurses and other clinicians caring for women with ASP.

PMID: 30738557 [PubMed - indexed for MEDLINE]

Long-term results after open repair of inflammatory infrarenal aortic aneurysms.

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Long-term results after open repair of inflammatory infrarenal aortic aneurysms.

J Vasc Surg. 2019 02;69(2):440-447

Authors: Wieker CM, von Stein P, Bianchini Massoni C, Rengier F, Böckler D, Geisbüsch P

Abstract
OBJECTIVE: The objective of this study was to investigate the long-term outcome after open repair of inflammatory infrarenal aortic aneurysms.
METHODS: A total of 62 patients (mean age, 68.9 ± 8.8 years; 91.9% male) undergoing open surgery for inflammatory aortic aneurysm from 1995 until 2014 in a high-volume vascular center were retrospectively evaluated. The patients' demographics, preoperative and postoperative clinical characteristics, imaging measurements, and procedural data were collected. Study end points were preoperative and postoperative sac diameter, evolution of periaortic fibrosis and development of hydroureteronephrosis detected by computed tomography (CT) scan, and mortality and morbidity after 30 days and at the time of maximum follow-up.
RESULTS: The mean abdominal aortic aneurysm diameter was 67.3 ± 16.7 mm. A total of 30 patients (48.4%) were asymptomatic, 27 patients (43.5%) were symptomatic, and 5 patients (8.1%) were treated for ruptured aneurysm. In 25 patients (40.3%), an aorta-aortic tube graft was implanted; in 37 patients (59.7%), an aortic bifurcation graft was used. Median operating time was 208 minutes (range, 83-519 minutes). Median aortic clamping time was 31 minutes (range, 14-90 minutes); in 25 patients (40.3%), suprarenal aortic cross-clamping was necessary. Hydroureteronephrosis was preoperatively diagnosed by CT scan in 16 patients (25.8%), with the need for a ureteral stent in 11 patients (17.7%). Aneurysm- and procedure-associated 30-day mortality was 11.3% (n = 7), with septic multiple organ failure in four patients and cardiac arrest in three patients. The overall perioperative complication rate was 33.9% (n = 21 patients). Median follow-up was 71.0 months (range, 0.2-231.6 months). At 1 year, 2 years, 4 years, and 6 years, overall survival was 83.4%, 79.6%, 79.6%, and 72.6%, respectively. Six patients (9.7%) required a reintervention during follow-up, predominantly aneurysm related and caused by aortoenteric fistula and graft infection (three of five patients). Median maximum thickness of preoperative perianeurysmal inflammation on CT was 10 mm (range, 2-22 mm), which decreased in 15 of 16 (94%) patients with available postoperative CT scans. Postoperative median thickness of perianeurysmal inflammation on CT was 6 mm (range, 0-13 mm). Hydroureteronephrosis persisted in two of nine (22.2%) patients at the end of follow-up.
CONCLUSIONS: Surgery in patients with inflammatory abdominal aortic aneurysms is associated with a substantial amount of perioperative complications. After surgery, the perianeurysmal inflammation decreases in most patients on follow-up CT. However, because the inflammatory process does not totally resolve, patients require lifelong surveillance for hydroureteronephrosis and development of aortoenteric fistulas.

PMID: 30503911 [PubMed - indexed for MEDLINE]

Predictors of positive 18F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica.

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Predictors of positive 18F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica.

Semin Arthritis Rheum. 2019 Feb;48(4):720-727

Authors: Prieto-Peña D, Martínez-Rodríguez I, Loricera J, Banzo I, Calderón-Goercke M, Calvo-Río V, González-Vela C, Corrales A, Castañeda S, Blanco R, Hernández JL, González-Gay MÁ

Abstract
OBJECTIVE: Polymyalgia rheumatica (PMR) is often the presenting manifestation of giant cell arteritis (GCA). Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan often discloses the presence of large vessel vasculitis (LVV) in PMR patients. We aimed to identify predictive factors of a positive PET/CT scan for LVV in patients classified as having isolated PMR according to well-established criteria.
METHODS: A set of consecutive patients with PMR from a single hospital were assessed. All of them underwent PET/CT scan between January 2010 and February 2018 based on clinical considerations. Patients with PMR associated to other diseases, including those with cranial features of GCA, were excluded. The remaining patients were categorized in classic PMR (if fulfilled the 2012 EULAR/ACR classification criteria at disease diagnosis; n = 84) or atypical PMR (who did not fulfill these criteria; n = 16). Only information on patients with classic PMR was assessed.
RESULTS: The mean age of the 84 patients (51 women) with classic PMR was 71.4 ± 9.2 years. A PET/CT scan was positive in 51 (60.7%). Persistence of classic PMR symptoms was the most common reason to perform a PET/CT scan. Nevertheless, patients with positive PET/CT scan often had unusual symptoms. The best set of predictors of a positive PET/CT scan were bilateral diffuse lower limb pain (OR = 8.8, 95% CI: 1.7-46.3; p = 0.01), pelvic girdle pain (OR = 4.9, 95% CI: 1.50-16.53; p = 0.01) and inflammatory low back pain (OR = 4.7, 95% CI: 1.03-21.5; p = 0.04).
CONCLUSION: Inflammatory low back pain, pelvic girdle and diffuse lower limb pain are predictors of positive PET/CT scan for LVV in PMR.

PMID: 29903537 [PubMed - indexed for MEDLINE]

Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center.

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Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center.

Semin Arthritis Rheum. 2019 Feb;48(4):714-719

Authors: Marroquín-Fabián E, Ruiz N, Mena-Zúñiga J, Flores-Suárez LF

Abstract
OBJECTIVES: Granulomatosis with polyangiitis-a primary systemic vasculitis-most frequent manifestations are respiratory. Airway disease can present with stenosis, and although subglottic stenosis (SGS) is well described, narrowing distal to the glottis has been more recently the focus of reporting. Our objectives, therefore, are to describe the frequency, characteristics, and treatment of tracheobronchial stenoses (TBS) in granulomatosis with polyangiitis (GPA) at our institution, a national referral center for respiratory diseases. Also, to identify factors associated with TBS development in GPA.
METHODS: We undertook a retrospective study of all identified TBS cases (n-29) in whom their demographics, clinical and paraclinical features, and treatment were analyzed with descriptive statistics. Comparisons between those who developed and did not develop TBS (n-79) were established with the χ2 test with Yates correction or two-tailed exact Fisher test for comparisons among groups with or without this complication.
RESULTS: Females were predominantly affected by TBS (76%). Most patients had limited GPA (n-20, 69%). TBS appeared at a mean of 29 months after diagnosis of GPA. Main symptoms were dysphonia, stridor, and dyspnea. All TBS patients had tracheal involvement and 12 (41%) additional bronchial stenoses. Other accompanying manifestations by organ/system were rhinosinusal (n-26, 87%), musculoskeletal (n-16, 55%), ocular (n-13, 45%), pulmonary (n-12, 41%), renal (n-8, 27%), mucocutaneous (n-5, 17%), neurological (n-4, 13%). At TBS diagnosis, 17 patients were PR3-ANCA and/or C-ANCA positive, while 9 were MPO-ANCA and/or P-ANCA positive; results on the remaining were either negative or ANCA were not performed at that time. Seventeen patients had simultaneous medical (8 with glucocorticoid therapy, 9 with immunosuppressants) and surgical therapy, while the rest only the latter at the time of TBS diagnosis. Relapses were frequent (one in 18 patients; two in 11, three in 9 and 2 individuals had ≥4 relapses). Factors positively associated with TBS were the presence of general and musculoskeletal symptoms and rhinosinusal disease, while those negatively associated were prednisone oral daily dose >10mgqd and azathioprine intake. The extent of disease, either generalized or limited was not associated with TBS development, nor were ANCA levels.
CONCLUSIONS: TBS are serious complications of GPA which can arise at any stage of the disease. Timely diagnosis plus optimal treatment and follow-up remain unmet needs.

PMID: 29891418 [PubMed - indexed for MEDLINE]

Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China.

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Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China.

Semin Arthritis Rheum. 2019 02;48(4):701-706

Authors: Chang DY, Li ZY, Chen M, Zhao MH

Abstract
OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA) directed to proteinase 3 (PR3) used to be considered the serologic marker for granulomatosis with polyangiitis (GPA). However, patients with myeloperoxidase (MPO)-ANCA positive GPA have been increasingly reported. The aim of this study was to analyze the clinical and pathological characteristics and outcome of Chinese patients with MPO-ANCA positive GPA.
METHODS: The clinical and renal histology data, renal outcomes, response to treatment, relapse and mortality were compared between patients with MPO-ANCA positive GPA and MPO-ANCA positive microscopic polyangiitis (MPA) as well as proteinase 3 (PR3)-ANCA positive GPA.
RESULTS: 455 patients with ANCA-associated vasculitis (AAV) were recruited in this study. 276/455 patients were classified as MPO-ANCA positive MPA, 4/455 patients were classified as PR3-ANCA positive MPA, 124/455 were MPO-ANCA positive GPA and 51/455 were PR3-ANCA positive GPA. Compared with MPO-ANCA positive MPA patients, MPO-ANCA positive GPA patients had significantly higher level of BVAS and milder renal lesion at diagnosis. The probability of developing ESRD was significantly higher in patients with MPO-ANCA positive MPA than MPO-ANCA positive GPA. MPO-ANCA positive GPA patients were likely to have relapse than MPO-ANCA positive MPA patients. Compared with PR3-ANCA positive GPA patients, MPO-ANCA positive GPA patients had significantly higher proportion of female, less constitutional symptoms and milder renal lesion at diagnosis.
CONCLUSIONS: Patients with MPO-ANCA positive GPA should be regarded as a unique subset of AAV. This subset of AAV patients had relatively milder renal injury. Although ANCA specificities play an important role in differentiating AAV, taking the disease type together to classify AAV may be more rational.

PMID: 29887327 [PubMed - indexed for MEDLINE]

Arterial lesions in giant cell arteritis: A longitudinal study.

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Arterial lesions in giant cell arteritis: A longitudinal study.

Semin Arthritis Rheum. 2019 02;48(4):707-713

Authors: Kermani TA, Diab S, Sreih AG, Cuthbertson D, Borchin R, Carette S, Forbess L, Koening CL, McAlear CA, Monach PA, Moreland L, Pagnoux C, Seo P, Spiera RF, Warrington KJ, Ytterberg SR, Langford CA, Merkel PA, Khalidi NA, Vasculitis Clinical Research Consortium

Abstract
OBJECTIVES: To evaluate large-vessel (LV) abnormalities on serial imaging in patients with giant cell arteritis (GCA) and discern predictors of new lesions.
METHODS: Clinical and imaging data from patients with GCA (including subjects diagnosed by LV imaging) enrolled in a prospective, multicenter, longitudinal study and/or a randomized clinical trial were included. New arterial lesions were defined as a lesion in a previously unaffected artery.
RESULTS: The study included 187 patients with GCA, 146 (78%) female, mean (±SD) age at diagnosis 68.5 ± 8.5 years; 39% diagnosed by LV imaging. At least one arterial lesion was present in 123 (66%) on the first study. The most frequently affected arteries were subclavian (42%), axillary (32%), and thoracic aorta (20%). In 106 patients (57%) with serial imaging, new arterial lesions were noted in 41 patients (39%), all of whom had a baseline abnormality, over a mean (±SD) follow-up of 4.39 (2.22) years. New abnormalities were observed in 33% patients by year 2; clinical features of active disease were present at only 50% of these cases. There were no differences in age, sex, temporal artery biopsy positivity, or disease activity in patients with or without new lesions.
CONCLUSIONS: In this cohort of patients with GCA, LV abnormalities on first imaging were common. Development of new arterial lesions occurred in patients with arterial abnormalities at first imaging, often in the absence of symptoms of active disease. Arterial imaging should be considered in all patients with GCA at diagnosis and serial imaging at least in patients with baseline abnormalities.

PMID: 29880442 [PubMed - indexed for MEDLINE]

Granulomatous spondylodiscitis: A case series with focus on histopathological features.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Granulomatous spondylodiscitis: A case series with focus on histopathological features.

J Spinal Cord Med. 2019 Apr 23;:1-6

Authors: Rammeh S, Romdhane E, Riahi H, Chebbi Y, Bouaziz MC, Achour W, Saidi LS, Benaissa HT, Ladeb MF

Abstract
OBJECTIVE: To report a series of Granulomatous Spondylodiscitis (GS) with focus on the histopathological features of the different forms of GS.
DESIGN: Case series.
SETTING: Pathology department of Charles Nicolle's Hospital of Tunisia Participants: This study included 57 patients diagnosed with GS. There were 44 (77.2%) female patients and 13 (22.8%) male patients (sex ratio = 0.28).
INTERVENTION: Not applicable.
OUTCOME MEASURES: Clinical, microbiological and histopathological features were assessed in this study.
RESULTS: Fifty-seven patients with GS were enrolled: 51 tuberculous spondylodiscitis (TS), 2 fungal spondylodiscitis (FS), 3 brucellar spondylodiscitis (BS) and 1 case of sarcoidosis. Granulomas with necrosis were seen in 38 (66.6%) cases: 36 TS and 2 FS, while granulomas without necrosis were observed in the remaining 19 cases: 15 TS, 3 BS and 1 sarcoidosis. In all cases of TS, granulomas were epithelioid type, associated with histiocytic type granulomas in 7 cases. Caseous necrosis was seen in 35 cases of TS and suppurative granuloma in one case. The 3 cases of BS exhibited non-necrotizing and histiocytic type granulomas. The 2 cases of FS showed histiocytic, epithelioid and necrotizing granulomas. Necrosis was mixed: suppurative and caseous in both cases of FS. Sarcoidosis was characterized with epithelioid type granulomas without necrosis.
CONCLUSION: Granuloma with caseous necrosis is highly suggestive of TS but does not rule out FS. Certain fungi can exhibit this type of necrosis as do tuberculosis species. Suppurative inflammation, although rare in TS, does exist. Histiocytic type granuloma without necrosis is suggestive of brucellosis.

PMID: 31012811 [PubMed - as supplied by publisher]

Elevated levels of periostin and TGF-β1 in the bronchoalveolar lavage fluid of patients with idiopathic eosinophilic pneumonia.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Elevated levels of periostin and TGF-β1 in the bronchoalveolar lavage fluid of patients with idiopathic eosinophilic pneumonia.

Asian Pac J Allergy Immunol. 2019 Apr 23;:

Authors: Katoh S, Matsumoto N, Tanaka H, Yasokawa N, Kittaka M, Kurose K, Abe M, Yoshioka D, Shirai R, Nakazato M, Kobashi Y

Abstract
BACKGROUND: Periostin is induced in bronchial epithelial cells and fibroblasts by various stimuli including interleukin (IL)13 and transforming growth factor (TGF)-β1, and is involved in allergic diseases such as asthma and atopic dermatitis, playing an important role in tissue remodeling and fibrosis. The role of periostin in the pathogenesis of eosinophilic lung diseases, however, is unclear.
OBJECTIVE: To examine the contribution of periostin to eosinophilic inflammation of the lung in humans, we evaluated periostin, IL-13, and TGF-β1 levels in the bronchoalveolar lavage fluid (BALF) of patients with eosinophilic pneumonia (EP).
METHODS: Periostin, IL-13, and TGF-β1 concentrations in the BALF were measured by enzyme-linked immunosorbent assay in patients with acute EP, chronic EP, idiopathic pulmonary fibrosis (IPF), and sarcoidosis. Further, we analyzed the relationship between periostin, IL-13, and TGFβ-1, levels and the number of inflammatory cells in the BALF.
RESULTS: The absolute number of eosinophils, and the periostin, IL-13, and TGF-β1 levels in the BALF were significantly higher in patients with EP than in patients with IPF and sarcoidosis. Concentrations of periostin significantly correlated with the concentrations of TGF-β1, but not those of IL-13, in the BALF of patients with EP. Periostin levels also significantly correlated with the absolute number of eosinophils in the BALF of patients with IPF, but not EP.
CONCLUSIONS: Our findings suggest that TGF-β1 might increase the production of periostin in the lungs of patients with EP. Periostin might contribute the pathogenesis of not only EP, but also IPF.

PMID: 31012597 [PubMed - as supplied by publisher]

[Hydrocephaly resulting from thrombophlebitis of the superior sagittal sinus due to osteitis of the cranial vault caused by Aspergillus fumigatus in an immunocompetent patient].

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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[Hydrocephaly resulting from thrombophlebitis of the superior sagittal sinus due to osteitis of the cranial vault caused by Aspergillus fumigatus in an immunocompetent patient].

Pan Afr Med J. 2018;31:97

Authors: Kouitcheu R, Oka DN, Varlet G

Abstract
This case study presents an unusual pathogenic association among several cranioencephalic lesions characterized by the association of osteitis of the cranial vault, due to Aspergillus fumigatus, with underlying thrombophlebitis complicated by intracranial hypertension resulting from hydrocephalus. The study involved a 43-year old HIV (human immunodeficiency virus) negative man with multi-recurrent infection of the frontal scalp. The patient was successfully treated with cerebrospinal fluid diversion (CFD), Ketoconazole and low molecular weight heparin. This study describes the different pathophysiological and therapeutic features of this exceptional pathogenic association.

PMID: 31011398 [PubMed - in process]

Subcutaneous nodules revealing systemic sarcoidosis: a case report.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Subcutaneous nodules revealing systemic sarcoidosis: a case report.

Pan Afr Med J. 2018;31:94

Authors: Tiresse N, Benataya H

PMID: 31011395 [PubMed - in process]

A Stab in the Dark: A Case Report of an Atypical Presentation of Giant Cell Arteritis (GCA).

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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A Stab in the Dark: A Case Report of an Atypical Presentation of Giant Cell Arteritis (GCA).

Geriatrics (Basel). 2018 Jun 29;3(3):

Authors: McCausland B, Desai D, Havard D, Kaur Y, Yener A, Bradley E, Patel HP

Abstract
Background: Giant cell arteritis/temporal arteritis (GCA) is an inflammatory condition that affects large to medium vessels such as the aorta and its primary branches. Patients classically present with fatigue, fever, headache, jaw claudication and in severe cases, may suffer either transient (amaurosis fugax) or permanent visual loss. The reference standard for diagnosis is the temporal artery biopsy (TAB) and the mainstay of treatment is with immunosuppression. Our patient JG, presented with a range of non-specific symptoms that mimicked generalised sepsis, but was ultimately diagnosed with GCA through effective, methodical multi-disciplinary team (MDT) work. Clinical case: JG, an 81 year old gentleman, presented acutely with a 3-4 weeks history of fatigue, lethargy, pyrexia and a marked inflammatory response suggestive of a sepsis but without a clear primary source or clinical features of vasculitis. His inflammatory markers were markedly raised although his erythrocyte sedimentation rate (ESR) was not elevated. He was initially treated for sepsis of unknown origin however, body imaging after admission suggested a possible infection around a previous aortic graft site. This was refuted in subsequent 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET/CT) scanning. Microbiological, parasitic, as well as autoimmune assays were unremarkable. He underwent a TAB which was diagnostic for GCA and as a result, was started on oral corticosteroids with immediate symptom relief. He was discharged and followed up on an outpatient basis. Conclusions: This case highlights how a vasculitis can present with a range of non-specific symptoms that may resemble a fever of unknown origin (FUO)/sepsis that can lead to a delay in making the correct diagnosis. It also highlights the importance of considering a diagnosis of vasculitis in patients who present with a FUO where there is no clear focus of infection. Delays in diagnosis and management of these conditions can potentially lead to significant irreversible morbidity.

PMID: 31011074 [PubMed]

Clinical Management of Cutaneous Adverse Events in Patients on Chemotherapy: A National Consensus Statement by the Spanish Academy of Dermatology and Venereology and the Spanish Society of Medical Oncology.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Clinical Management of Cutaneous Adverse Events in Patients on Chemotherapy: A National Consensus Statement by the Spanish Academy of Dermatology and Venereology and the Spanish Society of Medical Oncology.

Actas Dermosifiliogr. 2019 Apr 19;:

Authors: Sanmartín O, Beato C, Suh-Oh HJ, Aragón I, España A, Majem M, Segura S, Gúrpide A, Botella R, Grávalos C

Abstract
Although the arrival of new chemotherapy drugs and combinations has brought progress in terms of cancer patient survival, they entail many adverse effects that can compromise treatment, and hence prognosis, of the disease. Cytostatic agents can cause dermatological toxicity, among other side effects. The most familiar adverse effect of chemotherapy is alopecia. Although not serious, this changes the outward appearance of cancer patients. Other adverse effects include hypersensitivity and photosensitivity reactions, hand-foot syndrome, epidermal necrolysis, recall reactions, scleroderma-like reactions, Raynaud's phenomenon, eccrine squamous syringometaplasia, neutrophilic eccrine hidradenitis, nail abnormalities, pigmentation changes and extravasation injuries. Onset of these adverse effects often causes dose reduction and/or delayed treatment, which can affect patient survival and quality of life. It is therefore important to prevent their occurrence and treat them promptly, which requires cooperation between medical oncologists and dermatologists. This article reviews chemotherapy-associated dermatological toxicity, along with its diagnosis and therapeutic management.

PMID: 31010573 [PubMed - as supplied by publisher]

A 17-Year-Old Girl With Unilateral Headache and Double Vision.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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A 17-Year-Old Girl With Unilateral Headache and Double Vision.

J Investig Med High Impact Case Rep. 2019 Jan-Dec;7:2324709619838309

Authors: Rodriguez-Homs LG, Goerlitz-Jessen M, Das SU

Abstract
Tolosa-Hunt syndrome is characterized by a painful ophthalmoplegia secondary to a granulomatous inflammation in or adjacent to the cavernous sinus. Magnetic resonance imaging will show enhancement of the cavernous sinus and/or the orbital apex. Although this syndrome is extremely rare in children, it should be a diagnostic consideration in patients presenting with painful ophthalmoplegia with variable involvement of cranial nerves II to VI. The differential diagnosis for unilateral cavernous sinus lesion is broad, including vascular lesions (cavernous sinus thrombosis), inflammatory processes (sarcoidosis, autoimmune), neoplastic processes (schwannoma, lymphoma), as well as infectious etiologies. We describe a pediatric patient presenting with neurological symptoms from a unilateral cavernous sinus magnetic resonance imaging abnormality and the thorough diagnostic approach to arrive at the diagnosis of Tolosa-Hunt syndrome.

PMID: 31010318 [PubMed - in process]

Celiac Disease Prevalence is Increased in Primary Sjögren's Syndrome and Diffuse Systemic Sclerosis: Lessons from a Large Multi-Center Study.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Celiac Disease Prevalence is Increased in Primary Sjögren's Syndrome and Diffuse Systemic Sclerosis: Lessons from a Large Multi-Center Study.

J Clin Med. 2019 Apr 19;8(4):

Authors: Bartoloni E, Bistoni O, Alunno A, Cavagna L, Nalotto L, Baldini C, Priori R, Fischetti C, Fredi M, Quartuccio L, Carubbi F, Montecucco C, Doria A, Mosca M, Valesini G, Franceschini F, De Vita S, Giacomelli R, Mirabelli G, Bini V, Gabrielli A, Catassi C, Gerli R

Abstract
Association of celiac disease (CD) with systemic autoimmune diseases (ADs) remains controversial. Awareness of CD in these patients is important to prevent complications, including lymphoproliferative disorders. We evaluated previously diagnosed CD prevalence in systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS) and systemic sclerosis (SSc) patients in comparison to 14,298 matched controls. All patients were screened for subclinical CD. Data from 1458 unselected consecutive SLE (580), pSS (354) and SSc (524) patients were collected. Previously biopsy-proven CD diagnosis and both CD- and AD-specific features were registered. All patients without previous CD were tested for IgA transglutaminase (TG). Anti-endomysium were tested in positive/borderline IgA TG. Duodenal biopsy was performed in IgA TG/endomysium+ to confirm CD. CD prevalence in AD was compared to that observed in 14,298 unselected sex- and age-matched adults who acted as controls. CD was more prevalent in pSS vs controls (6.78% vs 0.64%, p < 0.0001). A trend towards higher prevalence was observed in SLE (1.38%, p = 0.058) and SSc (1.34%, p = 0.096). Higher CD prevalence was observed in diffuse cutaneous SSc (4.5%, p ≤ 0.002 vs controls). Subclinical CD was found in two SLE patients and one pSS patient. CD diagnosis usually preceded that of AD. Primary SS and SSc-CD patients were younger at AD diagnosis in comparison to non-celiac patients. Autoimmune thyroiditis was associated with pSS and CD. CD prevalence is clearly increased in pSS and diffuse SSc in comparison to the general population. The association of CD with diffuse but not limited SSc may suggest different immunopathogenic mechanisms characterizing the two subsets. CD screening may be considered in pSS and diffuse SSc in young patients, particularly at the time of diagnosis.

PMID: 31010199 [PubMed]

Practical suggestions on intravenous iloprost in Raynaud's phenomenon and digital ulcer secondary to systemic sclerosis: Systematic literature review and expert consensus.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Practical suggestions on intravenous iloprost in Raynaud's phenomenon and digital ulcer secondary to systemic sclerosis: Systematic literature review and expert consensus.

Semin Arthritis Rheum. 2019 Feb;48(4):686-693

Authors: Ingegnoli F, Schioppo T, Allanore Y, Caporali R, Colaci M, Distler O, Furst DE, Hunzelmann N, Iannone F, Khanna D, Matucci-Cerinic M

Abstract
BACKGROUND: Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud's phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3-5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials.
METHODS: A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise.
RESULTS: Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0ng/kg/min according to patient tolerability with a frequency depending on the indication.
CONCLUSIONS: Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials.

PMID: 29706243 [PubMed - indexed for MEDLINE]

Imatinib: A New Tool for the Management of Chronic Sclerodermatous Graft-Vs-Host Disease.

Actualités En Médecine Interne - mer, 24/04/2019 - 14:17
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Imatinib: A New Tool for the Management of Chronic Sclerodermatous Graft-Vs-Host Disease.

Actas Dermosifiliogr. 2018 Apr;109(3):200-201

Authors: Ferrándiz-Pulido C

PMID: 29429550 [PubMed - indexed for MEDLINE]

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease; +17 new citations

Actualités En Médecine Interne - mar, 23/04/2019 - 18:59

17 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease

These pubmed results were generated on 2019/04/23

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease; +17 new citations

Actualités En Médecine Interne - mar, 23/04/2019 - 15:03

17 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease

These pubmed results were generated on 2019/04/23

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Disease of the past re-emerging in modern Australian society.

Actualités En Médecine Interne - lun, 22/04/2019 - 12:22

Disease of the past re-emerging in modern Australian society.

BMJ Case Rep. 2019 Apr 20;12(4):

Authors: Uddin MA, Robson C, Dotel R

Abstract
A 28-year-old man with fever, atraumatic lower limb pain and rash was noted to have multiple areas of ecchymosis involving both lower limbs. He was anaemic and also had a grossly swollen left leg. Differential diagnoses of compartment syndrome, vascular tear, platelet and clotting factor disorders, vasculitis and myositis were ruled out. Scurvy was only considered after failing to reach a diagnosis. A dietary history revealed consumption of a restricted diet with no fresh fruits or vegetables. Diagnosis was supported by an undetectable vitamin C level in blood and a rapid improvement of symptoms on oral vitamin C replacement. Prevalence of vitamin C deficiency in developed countries is also discussed.

PMID: 31005867 [PubMed - in process]

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