Although the chest pain of acute aortic dissection is widely recognised, less consideration has been given to pain associated with other aortic pathologies. In light of contemporary concepts in aortic pathology we would like to present the pathology of a new cardiovascular syndrome—acute aortic syndrome (AAS).1 

This syndrome embraces a heterogeneous group of patients with a similar clinical profile that includes penetrating atherosclerotic aortic ulcer, intramural aortic haematoma, and the classic aortic dissection (fig 1). The physiopathological mechanism that precipitates the appearance of each of these entities is different. However, occasionally some patients exhibit several or all of these lesions, demonstrating the existence of a link between them. In such cases it is difficult to know which was the initiating event.



Figure 1 
Acute aortic syndrome (AAS). Arrows indicate the possible progression of each of these aortic lesions.



AAS is characterised clinically by aortic pain in a patient with a coexisting history of hypertension. In acute coronary syndromes, the existence of a typical chest pain that, since Heberden, has been called angina pectoris is well recognised. Likewise, in AAS chest pain is characteristic and has been called “aortic pain”. The recognition of pain associated with these progressive aortic lesions is of paramount importance. A severely intense, acute, searing or tearing, throbbing, and migratory chest pain denotes that the patient may have an AAS. Anterior chest, neck, throat, and even jaw pain is related to involvement of the ascending aorta, whereas back and abdominal pain more often indicates that the affected segment is the descending aorta.2 The chest pain and clinical presentation of patients with penetrating aortic ulcer and intramural aortic haematoma is similar to that of classic aortic dissection. Severe, acute chest pain consistent with AAS may occur in patients with pronounced aortic root dilatation but without histopathologic evidence of …

https://heart.bmj.com/content/heartjnl/85/4/365.full.pdf

Acute aortic syndrome

To determine the prevalence, clinical and radiological characteristics of spondyloarthropathy (SpA) in patients with inflammatory bowel disease (IBD), to assess the association between HLA B27 and B51 and the extraintestinal symptoms and to evaluate whether IBD is associated with Behcet’s disease (BD). One hundred and sixty-two consecutive adult patients with established diagnosis of IBD as either Crohn’s disease (CD) or ulcerative colitis (UC) were evaluated. All the patients including those previously diagnosed with or without SpA had a complete rheumatologic examination and they were evaluated according to the European Spondyloarthropathy Study Group (ESSG) criteria for SpA and The International Study Group for Behcet’s disease criteria for BD. The demographic and clinical data were recorded on a standardized form. The radiographies were obtained in all the patients and computed tomography (CT) was performed in the patients with suspected pelvic radiographies and/or low back pain in the physical examination. Radiological evaluation was made according to the Modified New York criteria. HLA B27, B51 and anti-neutrophile cytoplasmic antigen (ANCA) were searched in all the patients. Of the 162 patients with IBD (mean age 41.48±11.63 years, male 60, female 102), 78 were CD and 84 were UC. The mean of the IBD duration was 54.92±50.32 months and SpA duration was 20.63±34.37 months. The prevalence of SpA and AS in IBD was 45.7 and 9.9%, respectively. Frequencies of SpA and AS, the difference between UC and CD were not significant. Spondylitis, enthesitis, peripheral arthritis, oral ulcer and uveitis were not different between UC and CD, but erythema nodosum was found significantly more common in the CD patients compared with UC patients (P=0.005). The duration of IBD and SpA was similar in both groups. As the IBD duration increased, the prevalence of SpA development decreased (rr=0.991, P=0.009). Of the IBD patients, 13.6% were asymptomatic for musculoskeletal manifestations of SpA and their sacroiliac radiographies and CTs showed grade 2 sacroiliitis. HLA B27, B51 and ANCA positivities were not different between the patients with UC and CD. HLA B27 was significantly more common in the patients with sacroiliitis, spondylitis, enthesitis, peripheral arthritis, erythema nodosum, uveitis (P<0.001) and oral ulcer (P=0.025). BD was diagnosed in none of the patients. ANCA positivity was found to be related with the presence of erythema nodosum and uveitis (P=0.001 and P=0.005). The prevalence of SpA and AS is higher in the prospectively evaluated patients with radiological studies than those in the previously published studies. There is a high prevalence of asymptomatic sacroiliitis in IBD. An early diagnosis of inflammatory arthritis in IBD patients may prevent a disability due to SpA and AS.

The prevalence of extraintestinal manifestations and HLA association in patients with inflammatory bowel disease

Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases.

Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.

Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature

We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.

Solitary fibrous tumor of the central nervous system: a 15-year literature survey of 220 cases (August 1996-July 2011).

Hemangiopericytoma in the central nervous system : A study of eight cases

Introduction. - L'association d'une maladie de Behcet et d'une maladie de Crohn est exceptionnelle et pose un probleme nosologique etant donne qu'elles ont plusieurs manifestations cliniques en commun. Observation. - Une patiente, âgee de 24 ans, presentait depuis 1996 une diarrhee chronique, des douleurs abdominales, une aphtose bipolaire et une polyarthrite. La coloscopie revelait une colite ulceree diffuse. L'etude histologique concluait a des granulomes epitheloides giganto-cellulaires sans lesions de vascularite. Le diagnostic d'une maladie de Crohn etait pose et la patiente etait traitee par des corticoides par voie orale. L'evolution clinique etait favorable. En novembre 1998, elle etait rehospitalisee pour de nouvelles diarrhees. L'examen objectivait une pseudofolliculite, une uveite anterieure et le test pathergique etait positif. Son groupe HLA etait B51. Le diagnostic d'une maladie de Crohn associee a une maladie de Behcet etait retenu. Elle etait traitee par de la prednisone orale associee a du cyclophosphamide en bolus mensuel avec une bonne evolution clinique. Actuellement, la patiente est asymptomatique avec un recul de 5 mois. Conclusion. - Bien que d'origine pathogenique differente, la maladie de Behcet et la maladie de Crohn peuvent coexister chez un meme patient.

Association d'une maladie de Behçet et d'une maladie de Crohn

Association concomitante d’une maladie de Kikuchi et d’un lupus érythémateux systémique. À propos d’une observation

We report the case of a special form of rhabdomyosarcoma called "lymphadenopathic" which was reported only two times in the literature. This lymphadenopathic alveolar RMS shows lymph nodes as clinical manifestation mimicking lymphoma with an unknown primary neoplasm. In the case we report, clinical examination, and radiological studies revealed superficial and deep located lymph nodes in association with bilateral pleural effusion. Conventional histological examination showed lymph node infiltration by a metastatic tumour having alveolar pattern. Some of tumour cells were round with multilobed off-centred nucleus and eosinophilic cytoplasm containing fibril material with longitudinal and transverse striation indicating a muscle nature of the tumour proliferation. The immunohistochemical study showed intense and diffuse immunoreactivity of tumour cells for vimentin and a discreet immunostaining by desmin. These findings were compatible with the diagnosis of lymph node metastasis by alveolar rhabdomyosarcoma.

Lymphadenopathic form of alveolar rhabdomyosarcoma: A case report.

Un cas de dirofilariose souscutanéee en Tunisie

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