Alexandre de Melo Kawassaki

Desaturation – distance ratio: a new concept for a functional assessment of interstitial lung diseases

INTRODUCTION: The functional evaluation has become increasingly important in the understanding and management of patients with interstitial lung diseases. The cardiopulmonary exercise test and the six‐minute walk test (6MWT), through their isolated variables, have been used to do this evaluation, with some limitations. OBJECTIVES: We proposed a new composite index (desaturation distance ratio using continuous peripheral oxygen saturation (SpO2) and the distance walked as a more reliable tool for doing a functional evaluation of these patients. METHODS: 6MWT was performed by interstitial lung diseases patients and controls. Analyzed parameters were walked distance and desaturation area (DAO2), obtained by taking the difference between maximal SpO2 possible (100%) and patients SpO2 every 2 seconds. desaturation distance ratio was calculated using the ratio between DAO2 and distance walked. RESULTS: Forty‐nine interstitial lung diseases patients and 11 control subjects completed the protocol. The mean (SD) age was 60 (12) years and 65 (9) years, respectively (p:NS). Data obtained from 6MWT showed a significant statistical difference between interstitial lung diseases patients and controls: mean walked distance (430 and 602 meters, respectively); SpO2 minimal maintained at least 10 seconds ‐ SpO2 min (85% and 94%, respectively), and median desaturation distance ratio (10 and 2.5, respectively). A correlation analysis, considering interstitial lung diseases patients, revealed the best correlation between desaturation distance ratio and DLco (r =  ‐ 0.72; p<0.001), being the correlation between SpO2 min and DLco of 0.61 (p<0.001) and among walked distance and DLco of 0.58 (p<0.05). CONCLUSION: Desaturation distance ratio is a promising concept and a more reliable physiologic tool to assess pulmonary diseases characterized by involvement of the alveolar‐capillary membrane, such as interstitial lung diseases.

Guidelines for the diagnosis of rheumatoid arthritis

Description of the evidence collection method A review of the scientific literature was performed with the Medline database. The search for evidence was based on actual clinical scenarios and used the following Medical Subject Headings (MeSH) terms: Arthritis, Rheumatoid, Diagnosis (Delayed Diagnosis OR Delay OR Early Rheumatoid Arthritis OR VERA), Prognosis, Criteria (American College of Rheumatology/European League Against Rheumatism OR ACR/EULAR OR classification), Comparative Study, Smoking (OR tobacco use disorder), Rheumatoid Factor, Anti-cyclic Citrullinated Peptide (or anti-CCP), HLA-DRB1 OR PTPN22 OR EPITOPE, extra-articular OR extraarticular OR systemic OR ExRA, Disease Progression, Radiography OR X RAY, ULTRASONOGRAPHY, and MAGNETIC RESONANCE Grades of recommendation and strength of evidence A: A Most consistent experimental and observational studies. B: B Less consistent experimental and observational studies. C: C Case reports (uncontrolled studies). D: D Opinion that is not substantiated by critical evaluation, based on consensus, physiological studies or animal models. Objective To formulate guidelines for the management of rheumatoid arthritis (RA) in Brazil, with a focus on diagnosis. The aim of the present document is to summarise the current position of the Brazilian Society of Rheumatology on this topic to orient Brazilian doctors, particularly rheumatologists, to RA diagnosis in our country.

Pulmonary arterial involvement leading to alveolar hemorrhage in lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause that affects women of reproductive age. The disorder is characterized by the proliferation of atypical smooth muscle cells (LAM cells) around airways, blood vessels, and lymphatics with cystic destruction of the lung.1-3 LAM may occur sporadically or may be associated with tuberous sclerosis complex (TSC).1 Clinically, patients may develop progressive dyspnea, cough, wheezing, spontaneous pneumothorax, chylothorax, and hemoptysis.3 The diagnosis is confirmed by the identification of diffuse thin-walled lung cysts via thoracic high resolution computed tomography (HRCT) that are associated with a positive tissue biopsy or by the presence of chylothorax, angiomyolipoma, or TSC.1,2 Acute respiratory failure that is secondary to diffuse alveolar hemorrhage is an extremely rare manifestation in LAM patients, with only two previous cases described. The mechanism responsible for the comorbid presentation of acute respiratory failure and diffuse alveolar hemorrhage, however, is unknown. Nonetheless, it is speculated to be secondary to the obstruction of pulmonary venules by LAM cell proliferation, thereby promoting pulmonary venous hypertension.4,5 In the present work, we describe a patient with a diffuse alveolar hemorrhage that led to acute respiratory failure and death. A diagnosis of LAM and pulmonary arterial disruption caused by LAM cells was confirmed during the autopsy.

Interpretation of autoantibody positivity in interstitial lung disease and lung-dominant connective tissue disease.

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

Immunoglobulin G4-related systemic sclerosing disease in a patient with sclerosing cholangitis, inflammatory pseudotumors of the lung and multiple radiological patterns: a case report

IgG4-related systemic sclerosing disease (IRSSD) is a new clinical entity that is characterized by elevated levels of serum IgG4; plasma cell infiltrates in tissues, including the pancreas, lung, liver, kidney and breast; and a good response to corticosteroid therapy.1-4 We report a case of a patient with sclerosing cholangitis and pulmonary inflammatory pseudotumors. The diagnosis was made twelve years after the onset of the initial symptoms. The aim of this report is to describe a case with different radiological patterns.

Adenopathy and Pulmonary Infiltrates in a Japanese Emigrant in Brazil

A 77-year-old Japanese man living in Brazil for 70 years presented with a 6-month history of progressive dyspnea, weight loss (30 lb), low-grade fever, dry cough, and dry mouth. His medical history revealed hypertension and type 2 diabetes. He was a 40-pack-year ex-smoker and had worked in agriculture. Physical examination revealed bibasilar inspiratory crackles and enlarged (1 cm in diameter), mobile, and nontender cervical, supraclavicular, axillary, and inguinal lymph nodes (LNs).

Pulmonary involvement in rheumatoid arthritis: evaluation by radiography and spirometry

Abstract Objective: To determine whether simple diagnostic methods can yield relevant disease information in patients with rheumatoid arthritis (RA). Methods: Patients with RA were randomly selected for inclusion in a cross-sectional study involving clinical evaluation of pulmonary function, including pulse oximetry (determination of SpO2, at rest), chest X-ray, and spirometry. Results: A total of 246 RA patients underwent complete assessments. Half of the patients in our sample reported a history of smoking. Spirometry was abnormal in 30% of the patients; the chest X-ray was abnormal in 45%; and the SpO2 was abnormal in 13%. Normal chest X-ray, spirometry, and SpO2 were observed simultaneously in only 41% of the RA patients. A history of smoking was associated with abnormal spirometry findings, including evidence of obstructive or restrictive lung disease, and with abnormal chest X-ray findings, as well as with an interstitial pattern on the chest X-ray. Comparing the patients in whom all test results were normal (n = 101) with those in whom abnormal test results were obtained (n = 145), we found a statistically significant difference between the two groups, in terms of age and smoking status. Notably, there were signs of airway disease in nearly half of the patients with minimal or no history of tobacco smoke exposure. Conclusions: Pulmonary involvement in RA can be identified through the use of a combination of diagnostic methods that are simple, safe, and inexpensive. Our results lead us to suggest that RA patients with signs of lung involvement should be screened for lung abnormalities, even if presenting with no respiratory symptoms.Objective: To determine whether simple diagnostic methods can yield relevant disease information in patients with rheumatoid arthritis (RA).Methods: Patients with RA were randomly selected for inclusion in a cross-sectional study involving clinical evaluation of pulmonary function, including pulse oximetry (determination of SpO2, at rest), chest X-ray, and spirometry.Results: A total of 246 RA patients underwent complete assessments. Half of the patients in our sample reported a history of smoking. Spirometry was abnormal in 30% of the patients; the chest X-ray was abnormal in 45%; and the SpO2 was abnormal in 13%. Normal chest X-ray, spirometry, and SpO2 were observed simultaneously in only 41% of the RA patients. A history of smoking was associated with abnormal spirometry findings, including evidence of obstructive or restrictive lung disease, and with abnormal chest X-ray findings, as well as with an interstitial pattern on the chest X-ray. Comparing the patients in whom all test results were normal (n = 101) with those in whom abnormal test results were obtained (n = 145), we found a statistically significant difference between the two groups, in terms of age and smoking status. Notably, there were signs of airway disease in nearly half of the patients with minimal or no history of tobacco smoke exposure.Conclusions: Pulmonary involvement in RA can be identified through the use of a combination of diagnostic methods that are simple, safe, and inexpensive. Our results lead us to suggest that RA patients with signs of lung involvement should be screened for lung abnormalities, even if presenting with no respiratory symptoms.

Tiotropium use and pulmonary function in patients with constrictive bronchiolitis

foi demonstrado que o tiotropio foi capaz de atenuar o remodelamento de vias aereas promovido pelo TGF-β. Alem disso, ja foi demonstrada a resposta aguda a associacao de salbutamol e tiotropio em portadores de bronquiolite constritiva (BC) secundaria a doenca do enxerto contra o hospedeiro apos transplante de medula ossea, sendo observada variacao significativa no VEF

Lymphadenopathy and fever in a chef during a stay in Europe

This case illustrates a rare presentation (as lymphadenopathy and fever) of one of the most common zoonotic diseases worldwide-brucellosis-in a 22-year-old Brazilian male (a chef) who had recently returned to Brazil after having lived in and traveled around Europe for one year. The histopathology, clinical history, and response to treatment were all consistent with a diagnosis of brucellosis, which was confirmed by PCR in a urine sample. We also review some aspects of brucellosis, such as the clinical features, diagnosis, and management.

Erratum of the erratum of “Guidelines for the diagnosis of rheumatoid arthritis”

Licia Maria Henrique da Motaa,*, Bóris Afonso Cruza, Claiton Viegas Brenola, Ivânio Alves Pereiraa, Lucila Stange Rezende-Fronzaa, Manoel Barros Bertoloa, Max Vitor Carioca Freitasa, Nilzio Antônio da Silvaa, Paulo Louzada-Juniora, Rina Dalva Neubarth Giorgia, Rodrigo Aires Corrêa Limaa, Ronaldo Adib Kairallab, Alexandre de Melo Kawassakib, Wanderley Marques Bernardoc, Geraldo da Rocha Castelar Pinheiroa