Pulmonary involvement in patients with early rheumatoid arthritis.

OBJECTIVES: To assess the pleuropulmonary changes in patients with early rheumatoid arthritis (RA), using high-resolution computed tomography (HRCT). METHODS: Forty-three non-smoking patients with early RA were included. The disease duration was<1 year, without previous treatment. Disease activity was assessed using the 28-joint indices score (DAS28). Hand and wrist X-rays were evaluated using Larsen's criteria. Pulmonary functional tests (PFTs) were performed in 32 patients. The patients and 18 non-smokers healthy individuals were assessed by plain chest X-ray (CXR) and HRCT of the lungs. RESULTS: HRCT revealed air trapping in 69% (25/36), bronchiectasis in 58% (25/43), bronchial wall thickening in 52% (22/43) and ground glass opacities (GGOs) in 35% (15/43) of the patients. Pleural thickening and effusion were observed in 11% (5/43). CXR was abnormal in one patient revealing a single pulmonary nodule. GGOs were the only HRCT sign observed exclusively in RA patients. All the other abnormalities were depicted in the control group at the same frequency as in the patients. However, the extent (as expressed by the HRCT score) of air trapping, bronchiectasis and bronchial wall thickening was significantly greater in the patients than in the control group (p<0.05). The PFTs were within normal values. DAS28, PFTs, and the Larsen score did not show any significant correlation with either each HRCT sign score separately or the total score. CONCLUSIONS: Lung abnormalities are frequently observed in patients with early RA on HRCT, even when CXR and PFTs are normal. Limited areas of GGOs were the abnormalities depicted exclusively in patients.

A single pulmonary rheumatoid nodule masquerading as malignancy.

We present a 54-year-old man, a heavy smoker, with clinical and laboratory evidence of familial hypercholesterolemia and an asymptomatic solitary pulmonary nodule (SPN) increasing in size on follow-up chest X-ray. Laboratory work-up revealed high titers of rheumatoid factor and the presence of acute phase reactants. Because of the patient's age and history of smoking, open lung biopsy was performed to rule out malignancy. The biopsy showed histological features compatible with a rheumatoid nodule. Identical features were noted in the histological examination of the subcutaneous nodule. Usually, rheumatoid nodules occur in patients with active, long-standing rheumatoid arthritis (RA) with other extra-articular manifestations. The presence of a single pulmonary rheumatoid nodule as the first manifestation of RA is extremely rare. Histologic proof is often required since pulmonary carcinoma can present an identical clinical and radiological pattern. The diagnostic work-up and the differential diagnosis of an SPN, particularly in a heavy smoker, is a common clinical request and remains a diagnostic challenge.

Amiodarone-induced acute lung toxicity in an ICU setting.

Amiodarone is a highly effective antiarrhythmic drug, albeit notorious for its serious pulmonary toxicity. The incidence of amiodarone-induced pulmonary toxicity (APT) appears to be 1% per year (1). We report a case of very acute APT in a man suffering from postoperative atrial fibrillation.

Mucoepidermoid carcinoma of the thymus: a case presentation and a literature review.

Mucoepidermoid carcinoma of the thymus is an unusual, but well-recognized variant of thymic malignant tumors. Its biologic behavior generally depends on the degree of differentiation and the amount of cellular atypia. High grade tumors can be aggressive neoplasms with a tendency to invade and develop metastases. We report on a case of a 53-year-old man, who presented chest discomfort, dyspnea, and weakness. As heart function tests were normal, the patient underwent radiologic examination, which showed a well-demarcated mass in the anterior mediastinum. Histologic examination of the surgically resected mass showed features of a mucoepidermoid carcinoma with associated infiltration of the pleural tissue. Postoperative radiotherapy and chemotherapy were performed, and the patient died two months after initial diagnosis. In case of the absence of metastatic disease or other common primary neoplasms of the thymus, the diagnosis of a mucoepidermoid thymic carcinoma should be taken into consideration, although this tumor is rare.

Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren's syndrome.

Sinus histocytosis with massive lymphadenopathy is a rare disease that has been described by Rosai and Dorfman. It is characterized by massive, cervical lymphadenopathy, with extranodal manifestations in about 40% of patients. It occurs as a distinct entity, never associated with other diseases, and in most cases the prognosis is good. Lymphadenopathy is also a frequent sign of patients with primary Sjogren's syndrome (SS), usually associated with disease activity or concurrent infection. However, excessive lymphadenopathy in SS patients is a sign of lymphoproliferative disorder development. In this report, we describe a patient with primary SS, and excessive lymphadenopathy and splenomegaly who developed Rosai-Dorfman disease, and we discuss the possible aetiopathophysiological mechanism linking these two entities.

Primary angiosarcoma of the lung and pleura.

A 46 year old male smoker was admitted for severe continuing hemoptysis. Chest-X-rays and chest computed tomography revealed nodular infiltrates and bilateral hemothorax. Fiberoptic bronchoscopy resulted to non-diagnostic cytological and microbiological findings. Open lung and pleural biopsies after right thoracotomy revealed epithelioid angiosarcoma and further staging assessment showed secondary brain and liver foci. The patient received several courses of chemotherapy but he died one month later. The clinical, radiological, pathological, histochemical and therapeutic aspects of the disease are discussed. Angiosarcoma, a rare tumour with poor prognosis should be taken into consideration in the differential diagnosis of hemoptysis.

Conventional MRI and magnetisation transfer imaging of tumour-like multiple sclerosis in a child.

Tumefactive multiple sclerosis is a rare entity in children. Differential diagnosis includes other mass lesions such as neoplasm and abscess. A case of tumefactive multiple sclerosis in a child is presented. The open-ring pattern of enhancement on conventional MRI and magnetisation transfer imaging was important for the initial diagnosis and the evaluation of the course of the disease.

T(2) relaxation rate of basal ganglia and cortex in patients with beta-thalassaemia major.

In thalassaemic patients, neurophysiological disturbances have been associated with high serum ferritin levels and desferrioxamine therapy. In the presence of a magnetic field, ferritin, the main iron storage protein, induces a preferential decrease of the T(2) relaxation time. The purpose of this study was to evaluate thalassaemic patients for brain iron deposition by assessing the T(2) relaxation rate (1/T(2)) of the grey matter. 41 thalassaemic patients (age range 8.5-44 years, mean 24 years) and 58 age- and sex-matched controls were included in the study. Current serum ferritin levels were obtained. The 1/T(2) values of the cortex (motor and temporal) (mean 0.0122 ms(-1), SD 0.0004), putamen (mean 0.0137 ms(-1), SD 0.0004) and caudate nucleus (mean 0.0132 ms(-1), SD 0.0003) were higher in patients compared with the controls (mean 0.0110 ms(-1), SD 0.0004; mean 0.0120 ms(-1), SD 0.0005; mean 0.0117 ms(-1), SD 0.0003, respectively) (p<0.001 for all parameters). No statistically significant differences were found in the globus pallidus. No correlation was found between 1/T(2) and serum ferritin. The higher values of 1/T(2) in the cortex, putamen and caudate nucleus of thalassaemic patients probably reflect a higher iron deposition. The lack of differences in 1/T(2) of the globus pallidus might suggest that even in thalassaemic patients iron cannot exceed a saturation level.

Magnetisation transfer imaging of the normal adenohypophysis: the effect of sex and age.

Magnetisation transfer (MT) techniques provide tissue contrast depending mainly on the concentration of macromolecules. Because many macromolecules are involved in the secretory activity of the pituitary gland, MT techniques might be useful in the study of pituitary gland disorders. Our purpose was to establish a quantitative database of the MT ratio (MTR) of the normal adenohypophysis and to see whether there are age- and sex-related differences. Using a three-dimensional gradient-echo sequence for MT we studied the adenohypophysis in 56 males and 61 females aged 7 months-77 years; postsaturation images were obtained using an on-resonance binomial prepulse. The images were normal in all but three patients, two with meningiomas, one with a schwannoma). Adenohypophyseal MTR increased in both sexes up to 19 years of age (r = 0.47 males, 0.39 females, P < 0.05). In men after 20 years and in women from 20-49 years MTR decreased (r = -0.68, P < 0.001, r = -0.46, P < 0.05, respectively). In women aged 50-59 years the MTR again increased slightly. The MTR in males was slightly higher at all ages except before 9 and after 50 years of age. These differences were not statistically significant except in the 40-49 year group (P < 0.05).

Pituitary gland height evaluated by MR in patients with beta-thalassemia major: a marker of pituitary gland function.

In transfusion-dependent beta-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect leading mainly to hypogonadotropic hypogonadism. Our purpose was to assess in these patients the height of the pituitary gland and to evaluate whether it represents a marker of pituitary gland function. In 29 patients with beta-thalassemia major and 35 age- and gender-matched controls the pituitary gland height was evaluated in a midline sagittal scan using a spin echo T1 -weighted (500/20 TR/TE) sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulin-like growth factor, and adrenal hormones. The pituitary gland height was lower in thalassemic patients with hypogonadotropic hypogonadism (n = 15) (mean 3.48; SD 0.46) than in the age- and gender-matched controls (mean 6.29; SD 0.77), (P < 0.001). No statistically significant difference was found between thalassemic patients without hormone dysfunction (n = 14) (mean 5.34; SD 1.52) and age- and gender-matched controls (mean 5.91; SD 1.06). We conclude that in thalassemic patients the pituitary gland height is an additional marker of pituitary gland function and might be useful in clinical management.

T2 relaxation rate as an index of pituitary iron overload in patients with beta-thalassemia major.

OBJECTIVE: In transfusion-dependent ss-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect, leading mainly to hypogonadotropic hypogonadism. Early detection and quantification of iron in the pituitary gland are of particular importance for successful treatment. The purpose of this study was to evaluate the T2 relaxation rate (1/T2) as a marker of pituitary siderosis. SUBJECTS AND METHODS: In 29 patients with ss-thalassemia major and 40 controls, we assessed the 1/T2 of the pituitary gland in a 1.5-T MR unit, using a multiecho spin-echo sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulinlike growth factor, and adrenal hormones. RESULTS: A positive correlation was found between the 1/T2 and the serum ferritin level (r = 0.73, p < 0.001). The 1/T2 was higher in patients (mean, 0.020 msec(-1); SD, 0.006) compared with that of controls (mean, 0.011 msec(-1); SD, 0.001; p < 0.001). The 1/T2 was higher in patients with hypogonadotropic hypogonadism (mean, 0.024 msec(-1); SD, 0.006) in comparison with that of patients without any pituitary dysfunction (mean, 0.017 msec(-1); SD, 0.004; p < 0.05). CONCLUSION: The T2 relaxation rate could be used as an index of pituitary iron overload, and it might be of value to monitor treatment with deferoxamine in patients with ss-thalassemia major.