Rui Cunha

Epiploic Appendagitis: An Entity Frequently Unknown to Clinicians-Diagnostic Imaging, Pitfalls, and Look-Alikes

OBJECTIVE Epiploic appendagitis is an ischemic infarction of an epiploic appendage caused by torsion or spontaneous thrombosis of the epiploic appendage central draining vein. When it occurs on the right side of the abdomen, it can mimic appendicitis and right-sided diverticulitis; whereas when it occurs on the left side of the abdomen, it is often mistaken for sigmoid diverticulitis. The purpose of this article is to review the diagnostic imaging of this entity. CONCLUSION Epiploic appendagitis is self-limited and spontaneously resolves without surgery within 5-7 days. Therefore, it is imperative for radiologists to be familiar with this entity.

Influence of emphysema distribution on pulmonary function parameters in COPD patients

ABSTRACT OBJECTIVE: To evaluate the impact that the distribution of emphysema has on clinical and functional severity in patients with COPD. METHODS: The distribution of the emphysema was analyzed in COPD patients, who were classified according to a 5-point visual classification system of lung CT findings. We assessed the influence of emphysema distribution type on the clinical and functional presentation of COPD. We also evaluated hypoxemia after the six-minute walk test (6MWT) and determined the six-minute walk distance (6MWD). RESULTS: Eighty-six patients were included. The mean age was 65.2 ± 12.2 years, 91.9% were male, and all but one were smokers (mean smoking history, 62.7 ± 38.4 pack-years). The emphysema distribution was categorized as obviously upper lung-predominant (type 1), in 36.0% of the patients; slightly upper lung-predominant (type 2), in 25.6%; homogeneous between the upper and lower lung (type 3), in 16.3%; and slightly lower lung-predominant (type 4), in 22.1%. Type 2 emphysema distribution was associated with lower FEV1, FVC, FEV1/FVC ratio, and DLCO. In comparison with the type 1 patients, the type 4 patients were more likely to have an FEV1 < 65% of the predicted value (OR = 6.91, 95% CI: 1.43-33.45; p = 0.016), a 6MWD < 350 m (OR = 6.36, 95% CI: 1.26-32.18; p = 0.025), and post-6MWT hypoxemia (OR = 32.66, 95% CI: 3.26-326.84; p = 0.003). The type 3 patients had a higher RV/TLC ratio, although the difference was not significant. CONCLUSIONS: The severity of COPD appears to be greater in type 4 patients, and type 3 patients tend to have greater hyperinflation. The distribution of emphysema could have a major impact on functional parameters and should be considered in the evaluation of COPD patients.

Acute fibrinous and organizing pneumonia: A report of 13 cases in a tertiary university hospital.

IntroductionAcute fibrinous and organizing pneumonia (AFOP) is a rare diffuse pulmonary disease, but it is not yet known whether it is a distinct form of interstitial pneumonia or simply a reflection of a tissue sampling issue. MethodsCross-sectional evaluation of clinical and radiological findings, treatments, and outcomes for patients with histologically confirmed AFOP at a tertiary university hospital between 2002 and 2015. ResultsThirteen patients (7 women, 53.8%) with a mean ± SD age of 53.5 ± 16.1 years were included. The main symptoms were fever (69.2%), cough (46.2%), and chest pain (30.8%). All patients presented a radiological pattern of consolidation and 5 (38.5%) had simultaneous ground-glass areas. Histology was obtained by computed tomography-guided transthoracic biopsy in 61.5% of cases and by surgical lung biopsy in the remaining cases. Several potential etiologic factors were identified. Eight patients (61.5%) had hematologic disorders and 3 had undergone an autologous hematopoietic cell transplant. Two (15.4%) had microbiologic isolates, 5 (38.4%) had drug-induced lung toxicity, and 2 (15.4%) were classified as having idiopathic AFOP. In addition to antibiotics and diuretics used to treat the underlying disease, the main treatment was corticosteroids, combined in some cases with immunosuppressants. Median survival was 78 months and 6 patients (46.2%) were still alive at the time of analysis. ConclusionOur findings for this series of patients confirm that AFOP is a nonspecific reaction to various agents with a heterogeneous clinical presentation and clinical course that seems to be influenced mainly by the severity of the underlying disorder.

Influência da distribuição do enfisema nos parâmetros de função pulmonar em pacientes com DPOC

A distribuicao do enfisema foi analisada em pacientes com DPOC, que foram classificados de acordo com um sistema de classificacao visual de cinco pontos a partir de achados de TC de torax. Avaliou-se a influencia do tipo de distribuicao do enfisema na apresentacao funcional e clinica da DPOC. Hipoxemia apos o teste da caminhada de seis minutos (TC6) foi tambem avaliada e a distância percorrida (DTC6) foi determinada.

Analysis of sarcoidosis in the Oporto region (Portugal)

BACKGROUND Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal. METHODS A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014. RESULTS We identified 409 patients with sarcoidosis (females, 58.9%; mean age at diagnosis, 38.9±13.4 years; smokers, 14.4%]. All the patients were diagnosed according to the ERS/ATS/WASOG consensus statement and 64.1% had evidence of noncaseating epithelioid cell granulomas in biopsy specimens. Bronchoalveolar lavage was performed as part of the diagnostic work-up in 289 patients and 90.2% had lymphocytosis (CD4/CD8 ratio ≥3.5 in 60.9% of cases). Exertion dyspnea, cough, and constitutional symptoms were the most common presenting symptoms; 10.1% of patients were asymptomatic, 22.8% had Löfgren syndrome, and 50.5% had extrathoracic involvement. Radiographic stages of disease according to the Scadding criteria were as follows: stage 0 (5.2%), stage I (33.7%), stage II (47.0%), stage III (8.4%), and stage IV (5.7%). Impaired respiratory function was observed in 45.6% patients and was mostly mild. Systemic treatment was administered in 58.6% of cases. Overall, 45.3% of patients experienced disease resolution. CONCLUSION The epidemiological and clinical characteristics of this cohort of patients with sarcoidosis from the Oporto region in northern Portugal revealed epidemiological and clinical characteristics that were generally similar to those described in other Western Europe populations and in the US ACCESS study. However, we found a higher proportion of patients who progressed to chronic forms.

Hepatic perivascular epithelioid tumor (PEComa). A case report

A 37-year-old female had liver nodules found in an abdominal ultrasound scan. The radiological features were suggestive of hepatocellular adenomas, although there were some atypical findings. Two years later, one of the nodules showed dimensional progression and intralesional hemorrhage. The patient underwent a left hepatectomy and the postoperative course was uneventful. The histological exam and the immunohistochemistry were consistent with the diagnosis of PEComa. The PEComa is a mesenchymal tumor rarely described in the liver. The preoperative diagnosis of PEComa is very difficult and, with this report, the authors intend to increase the limited knowledge relating to natural history and optimal treatment of this rare condition.

Evaluation of clinical presentation and outcome of patients with respiratory bronchiolitis-associated interstitial lung disease.

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Peripheral inflammatory pseudotumor of the liver and extensive thrombosis of the portal venous system in a child

We report a case of a peripheral inflammatory pseudotumor of the right lobe of the liver with extensive thrombosis of the portal venous system in a 9-year-old boy. Local thrombosis of the portal vein is a known complication of the inflammatory pseudotumor, especially in the hepatic hilum. The extent of the thrombosis in this case was unexpected, considering the peripheral location and the benign nature of the lesion. To our knowledge, thrombosis of this degree has not been described.

Unusual Merkel cell carcinoma in a renal transplant recipient: case report and review of the literature

Merkel cell carcinoma (MCC) is an uncommon and aggressive neuroendocrine primary skin cancer known for its high propensity for local recurrence and distant metastases. There is no consensus regarding the optimal therapeutic approach, and the relative roles of surgery, radiotherapy and chemotherapy are still controversial. MCC generally affects elderly patients in areas of actinically damaged skin. Several studies suggest that a more aggressive course is observed in immunocompromised patients such as organ transplant recipients. Clinically, MCC usually appears as a rapidly growing, dome-shaped and solitary nodule. More than half of these tumours occur in the head and neck, and can become a challenge for the plastic and reconstructive surgeon. An unusual case of this cutaneous malignancy is presented in a renal transplant recipient. The tumour was located in the medial canthus of the eye. Tumour resection was performed and a reverse flow pre-auricular island flap and a forehead flap were used for reconstruction.

Monitoring Crohn’s disease activity: endoscopy, fecal markers and computed tomography enterography:

Background: The treatment goal of Crohn’s disease (CD) has moved towards achieving mucosal healing, resolution of transmural inflammation, and normalization of biomarkers. The purpose of this study was to evaluate how well computed tomography enterography (CTE) and fecal calprotectin (FC) correlated with endoscopic activity in newly diagnosed patients with CD and after 1 year of therapy. Methods: Consecutive patients with newly diagnosed CD were evaluated by endoscopy, CTE, and FC at diagnosis and 12 months after beginning immunosuppression. Endoscopic severity was assessed using the Simplified Endoscopic Score for Crohn’s Disease (SES-CD). Biomarkers, clinical indexes, and FC were recorded on the day of ileocolonoscopy at diagnosis and 1 year after diagnosis. We adapted a CTE score for disease activity based on radiological signs of inflammation (i.e. mural thickness, mural hyperenhancement, mesenteric fat proliferation, mesenteric fat densification, comb sign, presence of strictures, fistulas, abscesses, ascites, and lymphadenopathy). Correlations between endoscopy, CTE, and FC were assessed using Spearman’s rank correlation. Results: A total of 29 patients (48% women; median age 30 (24.5–35.5) years) were included in this prospective cohort. CTE findings significantly correlated with endoscopic findings. Endoscopic remission (ER) at 1-year follow up significantly correlated with improvement in mural hyperenhancement (p = 0.004), mesenteric fat densification (p = 0.001), comb sign (p = 0.004), and strictures (p = 0.008) in CTE. None of the CTE findings improved in patients without ER. FC correlated with SES-CD (rs = 0.696, p < 0.001) and with CTE features of inflammation (rs = 0.596, p < 0.001). A cut-off of 100 µg/g predicted ER with 92% sensitivity, 65% specificity, and 83% accuracy (area under curve 0.878, p < 0.001). Conclusions: CTE findings and FC levels correlated with endoscopic activity in CD both at diagnosis and at 1-year follow up. These two noninvasive markers of disease activity may be used as an alternative to endoscopy to monitor disease response to therapy.