RESUMEN
Sarcoidosis is a multiorgan inflammatory disease that rarely involves the musculoskeletal system. A typical radiographic presentation is only noted with phalangeal lesions in the hands and feet, and other skeletal sites of sarcoidosis are a diagnostic imaging challenge [1]. We describe two cases of patients with sarcoidosis in whom pathologic bone marrow lesions were diagnosed on MRI scans. The magnetic resonance findings were non-specific and metastatic lesions or multiple myeloma were suspected. The case analysis serves to point to limitations of imaging studies in diagnosing bone sarcoidosis and underline the importance of cooperation between the radiologist and the clinician. The role of magnetic resonance imaging in the diagnostic algorithm for bone sarcoidosis should mostly focus on locating lesions, indicating biopsy sites and follow-up of abnormalities.
Asunto(s)
Enfermedades Óseas/diagnóstico por imagen , Enfermedades Óseas/fisiopatología , Neoplasias de la Médula Ósea/diagnóstico por imagen , Neoplasias de la Médula Ósea/fisiopatología , Angiografía por Resonancia Magnética , Sarcoidosis/diagnóstico por imagen , Sarcoidosis/fisiopatología , Humanos , Sarcoidosis/diagnósticoRESUMEN
INTRODUCTION: The first-line therapy in chronic sarcoidosis, according to WASOG/ATS/ERS recommendations, is GCS. This therapy is associated with significant adverse effects and finally does not alter the natural history of the disease. The objective of our study was to evaluate the efficacy and safety of monotherapy with MTX, as an alternative to GCS, in progressive pulmonary sarcoidosis. MATERIAL AND METHODS: An open prospective real-life, single-centre trial was performed on 50 patients with biopsy proven sarcoidosis, 28M and 22F, mean age 45.55 ± 8.9 years. The average duration of disease before MTX therapy was 12.34 ± 20.49 years, GCS therapy in the past was applied in 41 patients. All patients received MTX (10 mg or 15 mg weekly) between 2004 and 2013 because of chronic progressive pulmonary sarcoidosis. Therapy was planned for 24 months. Patients underwent regular clinical evaluation, pulmonary function assessment, exercise ability testing (6MWT), and chest radiography for therapy effectiveness every six months and side effects monitoring every 4-6 weeks. Forty-nine patients were included for statistical analysis of treatment efficacy. They were retrospectively allocated to "MTX responder" group if an improvement of 10% of FEV1, FVC, TLC, or 15% of DLCO from the initial value was documented for at least one parameter or "non-responders" if the patient did not meet the above-mentioned criteria. RESULTS: Duration of treatment ranged from 6 to 24 months, mean time 60.75 ± 34.1 weeks. For the whole cohort significant improvement after MTX therapy was observed for minimal SaO2 (%) (p = 0.043) and for decrease of DSaO2 (%) (p = 0.048) in six-minute walk test. The results were significantly better for patients treated with 15 mg than for those treated with 10 mg weekly and for those who obtained a greater total amount of MTX during therapy. Significant difference of DLCO%pred was observed after six months of MTX therapy between groups treated 15 mg vs 10 mg weekly (73.27 ± 12.7% vs. 63.15 ± 16.4%, p = 0.03). Twenty-five patients (55%) met the criteria of "MTX responders" group. Patients who responded well to treatment had significantly lower TLC and FVC initial values comparing to "MTX non-responders". After treatment the only significant difference in PFT between groups was noted for DLCO%pred. Eleven patients (22%) stopped the treatment due to adverse events of MTX, mild hepatic abnormalities were observed in ten patients (20%), and concomitant infection was found in four patients. There were no patients with a fatal outcome. CONCLUSIONS: MTX as a single agent in the treatment of sarcoidosis has proved to be a safe and effective steroid alternative. Selected patients with chronic pulmonary sarcoidosis experience definite PFT improvements after MTX treatment. There is need to search for predictors of MTX treatment effectiveness.
Asunto(s)
Fármacos Dermatológicos/administración & dosificación , Metotrexato/administración & dosificación , Sarcoidosis Pulmonar/tratamiento farmacológico , Administración Oral , Adulto , Anciano , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios ProspectivosRESUMEN
A 62-year-old female suspected of malignant disease underwent a splenectomy that revealed noncaseating granulomas in the histological specimen. Chest X-ray (CXR) and lung CT scans suggested sarcoidosis stage II. TBLB showed noncaseating granulomas. A diagnosis of sarcoidosis was made. Initially no treatment was needed as partial remission on CXR and normal lung function were observed. During the follow up she underwent open lung biopsy and axillary lymph node biopsy because of radiological progression with presence of CXR opacities imitating metastases and recurrent lymphadenopathy. No malignant cells were found. Spontaneous partial resolution of disseminated changes on the CXR was observed. Because of progressive deterioration in lung function and the clinical course of the disease strongly suggesting the progression of systemic sarcoidosis, the patient was given steroid treatment, which initially resulted in partial remission of pulmonary disseminated changes, peripheral lymphadenopathy and improvement in lung function test. Eight months later severe deterioration in general condition, anaemia, leukocytosis, hypoxemia, massive hepatomegaly and recurrence of general lymphadenopathy along with progression of disseminated changes were found. She died before the final diagnosis was established. Post-mortem examination showed a nodal marginal zone B-cell lymphoma with monocytoid B-cells, according to WHO classification. The malignant cells were found in the jugular, mediastinal, paratracheal, paragastric, paraintestinal and retroperitoneal lymph nodes and they infiltrated the lungs, pleura, liver, thyroid gland and pancreas. No sarcoid granulomas were found in the autopsy.
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Linfoma/diagnóstico , Sarcoidosis Pulmonar/diagnóstico , Biopsia , Resultado Fatal , Femenino , Granuloma/patología , Granuloma/cirugía , Humanos , Pulmón/patología , Linfoma/terapia , Persona de Mediana Edad , Sarcoidosis Pulmonar/patología , Sarcoidosis Pulmonar/terapia , Bazo/patología , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/terapia , SíndromeRESUMEN
Yellow nail syndrome (YNS) is a condition characterized by yellow-green coloration of nails, respiratory manifestations and lymphoedema. This article presents 52-year-old patient with membranous glomerulonephritis, hospitalized at the National Tuberculosis and Lung Diseases Research Institute in Warsaw, because of suspected allergic aspergillosis. Based on clinical and radiological evaluation the diagnosis of YNS was established. Treatment of renal disease did not affect the course of yellow nail syndrome. During the two-year follow-up, despite stable renal parameters we observed the progression of respiratory manifestations (bronchiectasis, pleural effusions).
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Glomerulonefritis Membranosa/complicaciones , Glomerulonefritis Membranosa/diagnóstico , Síndrome de la Uña Amarilla/complicaciones , Síndrome de la Uña Amarilla/diagnóstico , Bronquiectasia/complicaciones , Bronquiectasia/diagnóstico , Progresión de la Enfermedad , Estudios de Seguimiento , Glomerulonefritis Membranosa/terapia , Humanos , Masculino , Persona de Mediana Edad , Derrame Pleural/complicaciones , Derrame Pleural/diagnóstico , PoloniaRESUMEN
Severe complications of lower respiratory tract infection in a patient with hereditary glucose-6-phosphate dehydrogenase (G-6-PD) deficiency may occur. The case of a 68-year-old man with hereditary glucose-6-phosphate dehydrogenase (G6PD) deficiency who developed severe haemolysis after community-acquired pneumonia is presented. G6PD deficiency in our patient was diagnosed during childhood. We observed complications of community-acquired pneumonia: empyema, haemolytic crisis and renal failure. Videopleuroscopy and pleural drainage were successfully performed. Community-acquired streptococcal pneumonia may also lead to haemolysis in G6PD deficient patients. Acute haemolysis, severe anaemia and renal insufficiency secondary to haemoglobinuria can be observed. Severe purulent complications of pneumonia in G6PD deficient patients may suggest granulocyte function impairment.
Asunto(s)
Deficiencia de Glucosafosfato Deshidrogenasa/complicaciones , Neumonía Bacteriana/complicaciones , Neumonía Bacteriana/diagnóstico por imagen , Anciano , Infecciones Comunitarias Adquiridas/complicaciones , Humanos , Masculino , RadiografíaRESUMEN
Three patients with exogenous lipoid pneumonia are presented. All of them had laryngectomy because of the cancer of larynx. In a period of time ranging from a few months up to a few years after the operation they started to have dyspnoea, cough, sometimes hemoptysis and slightly elevated temperature. The chest X-ray revealed massive opacities in the lower zones of both lungs suggesting lung cancer or metastases of the laryngeal cancer. HRCT showed ground glass lesions. Histological examination of the lungs detected accumulation of multiple macrophages with vacuolated foamy cytoplasms in the alveoli. Neoplastic disease was excluded. Additional data from the patients history revealed that all of them were inhaling or administering mineral oil-containing products through the tracheostomy. Exogenous lipoid pneumonia was diagnosed. Patients were asked to stop inhalations with the mineral oil.
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Emolientes/efectos adversos , Neoplasias Laríngeas/cirugía , Laringectomía , Aceite Mineral/efectos adversos , Neumonía Lipoidea/inducido químicamente , Anciano , Humanos , Masculino , Persona de Mediana Edad , Neumonía Lipoidea/diagnóstico por imagen , Neumonía Lipoidea/patología , Tomografía Computarizada por Rayos XRESUMEN
We report the case of a 20-year-old female with disseminated Mycobacterium avium disease involving bones, lungs and brain. She was completely healthy up until the present illness and had been vaccinated with BCG in infancy without complications. Mycobacteriosis progressed in spite of treatment with antituberculous drugs and was controlled only after addition of interferon-gamma subcutaneously. A homozygous hypomorphic I87T mutation was found in the gene encoding the ligand-binding chain of the IFN-gamma receptor (IFNgammaR1). This mutation is the only known recessive hypomorphic lesion in IFNGR1 and had been reported before in only 1 child with curable BCG infection and his sibling with primary tuberculosis. Our report illustrates the clinical heterogeneity of patients sharing exactly the same form of partial recessive IFNgammaR1 deficiency. A diagnosis of partial recessive IFNgammaR1 deficiency should be contemplated in adults with unexplained environmental mycobacterial diseases.
Asunto(s)
Síndromes de Inmunodeficiencia/complicaciones , Mycobacterium avium/aislamiento & purificación , Receptores de Interferón/deficiencia , Tuberculosis/complicaciones , Adulto , ADN/genética , Diagnóstico Diferencial , Exones , Femenino , Expresión Génica , Humanos , Síndromes de Inmunodeficiencia/diagnóstico , Síndromes de Inmunodeficiencia/metabolismo , Interferón gamma/deficiencia , Imagen por Resonancia Magnética , Reacción en Cadena de la Polimerasa , Receptores de Interferón/genética , Tuberculosis/diagnóstico , Tuberculosis/microbiología , Receptor de Interferón gammaRESUMEN
Ljmphocytic interstitial pneumonia (LIP) is a rare form of interstitial pneumonia. It can occur as a idiopathic disease however most frequently associated with other diseases, particularly Sjogren syndrome. We present a 58 years old woman, with primary Sjögren syndrome who developed after 7 years interstitial lung disease. LIP was diagnosed on the basis of histological examinations of specimens obtained by open lung biopsy. Diffuse infiltrations composed of small polyclonal lymphocytes, plasma cells with reactive follicules were found. Infiltrates observed in bronchial and bronchiolar walls expanded into interlobular and alveolar septae. Prednisone in a dose of 40 mg per day was introduced and clinical, radiological, spirometric and gasometric improvement was observed. The dose of prednisone was gradually reduced but is still continued.