Revista Colombiana de Reumatologia最新文献

Introduction

Alveolar hemorrhage syndrome can be secondary to multiple autoimmune disorders. The objective is to describe three diffuse alveolar hemorrhage (DAH) cases secondary to anti-synthetase syndrome (ASSD).

Presentation of the case

Three cases of ADH secondary to ASSD are described: one positive to anti-PL7, another positive to anti-PL12, and the last patient with double positivity to anti-Jo1 and anti-OJ. The patients presented improvement after receiving immunosuppressive treatment.

Discussion

The evolution with therapeutic response and resolution of DAH supports the conclusion that ASSD is a potentially treatable cause of DAH and should be considered within the differential diagnosis in diagnosing DAH.

Conclusion

The described cases contribute to the knowledge of DAH, where ASSD should be considered in diagnosing DAH.

Introduction/Objective

To describe the safety and response to treatment with rituximab (RTX), estimating its impact on the health state utility (HSU) of patients with refractory lupus nephritis (LN) treated in referral centres in several cities in Colombia.

Materials and methods

A registry-based follow-up study. Patients aged between 16 and 75 years, who were refractory to first-line management and had ISN/RPS class III-IV (± V) LN, were included. Our primary outcome was total or partial response to treatment; secondary outcomes were HSU measured with the EQ-5D-3L, and safety of treatment with RTX. The impact analysis of response to RTX on HSU were performed by mean difference estimated by robust regression.

Results

Forty-six patients (44 women) were included, with a median age of 34 years (IQR = 13), the median SDI was 1 (IQR = 1) and the median activity measured by SLEDAI was 4.5 (IQR = 5.9). Response to RTX was observed in 27 (58.7%) patients. Adjusted for SLEDAI and co-interventions, the patients who responded to RTX obtained a higher mean HSU by 0.162 (95% CI: 0.006-0.317). Which is equivalent to 1.9 (95% CI: 0.2-3.8) more months lived in ideal health conditions for each year with refractory LN. In 54.3% of the patients, RTX had adequate safety.

Conclusion

From the patient's perspective, the response to treatment with RTX in patients with refractory LN implies a significant impact on their quality of life.

Introduction

Anti-synthetase syndrome is a recently characterized entity whose morbidity and mortality are mainly determined by interstitial lung involvement. For this reason, it is considered important to identify the association between the presence of anti-synthetase antibodies and the presence and putatively, the development of a specific radiological pattern of interstitial lung disease.

Objective

To determine the association between the antibodies present at the time of diagnosis of anti-synthetase syndrome and the presence of interstitial lung disease.

Materials and methods

Systematic review of the literature and meta-analysis. The search strategy was carried out in: EMBASE, LILACS, PUBMED, CENTRAL (Cochrane), and Grey Literature. The primary outcomes were the detection of the different radiological patterns of interstitial lung disease, and the reported specific anti-synthetase antibody.

Results

One hundred seventy-six patients were identified; Jo-1 in combination with NSIP was the most frequent pattern. Quantitative analysis suggests that PL-7 expression is associated with the presence of UIP and NSIP. For obstructive pneumonitis, a relationship was observed with the presence of anti EJ, while the expression of PL-7 was negatively associated. Also, EJ had a negative association with the presence of NSIP. The observed associations were corroborated with the subgroup analysis carried out using the two retrospective observational studies identified.

Conclusion

Despite the limitations, PL-7 and EJ showed significant associations with the presence of specific patterns of interstitial lung disease. Jo-1 did not have a significant specific association. Studies of higher methodological quality are required to generate recommendations that affect clinical practice.

Interstitial lung disease is a common complication of Sjögren's syndrome that can occur at diagnosis or during follow-up. To detect it, complete pulmonary function studies should be performed, including spirometry, measurement of lung volumes, and DLCO, with the latter being the most sensitive parameter for detecting the presence of the disease. High-resolution computed tomography is essential for the study. Sixty percent of patients present a single tomographic pattern, with non-specific interstitial pneumonia being the most frequent pattern, followed by usual interstitial pneumonia pattern. Mortality is high, being higher in those with lower forced vital capacity, lower DLCO, and higher fibrosis score on chest computed tomography. Currently, there are two international guidelines for the treatment of pulmonary manifestations of Sjögren, but recommendations are based on low-quality scientific evidence. A stepwise approach is suggested, initially with glucocorticoids, then immunosuppressants, and in refractory or severe cases, considering other agents such as rituximab. The use of antifibrotic medication is recommended in patients who develop progressive pulmonary fibrosis as defined by current criteria. It is important to bear in mind that although non-specific interstitial pneumonia is considered a pattern where inflammation predominates, there may be progression to progressive pulmonary fibrosis in some cases. Lung transplantation and oxygen therapy may be options for selected patients. The relevance of an interdisciplinary team approach to achieve adequate diagnosis and treatment of patients is highlighted.

Interstitial lung disease (ILD) is a common and serious manifestation of autoimmune rheumatic diseases. While the prevalence of ILD differs among the individual autoimmune rheumatic diseases, ILD remains an important cause of morbidity and mortality in systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, primary Sjögren's disease, rheumatoid arthritis, and idiopathic inflammatory myositis. The present review summarizes recent literature on autoimmune-associated ILD with a focus on screening and monitoring for ILD progression. Reflecting on the currently available evidence, the authors propose a guideline for monitoring for progression in patients with newly diagnosed autoimmune-associated ILD. This review also highlights clinical and biological predictors of progressive pulmonary fibrosis and describes opportunity for further study in the rapidly evolving area of rheumatology and pulmonology.

Interstitial lung disease occurs with high frequency as an initial or late manifestation of multiple rheumatic diseases, including systemic sclerosis, idiopathic inflammatory myopathies, rheumatoid arthritis, systemic lupus erythematosus, primary Sjögren's syndrome and antineutrophil cytoplasmic antibody-associated vasculitis. Thus, the rheumatologist must be clear about certain concepts of pneumology, including the evaluation of lung function tests, the approach to radiological patterns observed on high-resolution computed tomography of the chest, and concepts such as interstitial pneumonia with autoimmune features. In this article, we present our approach to patients with interstitial lung disease, in whom an autoimmune etiology is suspected. We propose a sequential diagnostic strategy, recognizing the importance of the multidisciplinary team and including the autoimmune perspective with emphasis on clinical and serological domains. Other diagnostic tools such as capillaroscopy and minor salivary gland biopsy are also considered. We also take a critical look at the latest guidelines for progressive pulmonary fibrosis, since it is essential that the rheumatologist understands these concepts that are vital in a multidisciplinary team.

This article will mention the essential aspects of managing ILD associated with systemic autoimmune diseases such as rheumatoid arthritis (RA) and inflammatory myopathies (IIM). The prognosis of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has recently improved because of tighter control of RA disease activity. This article presents recent evidence of the effect of methotrexate on RA-ILD, which is associated with a better prognosis. The available alternatives include the use of anti-fibrotic drugs. In managing interstitial lung disease related to anti-synthetase syndrome (ASSD-ILD) and anti-MDA5-associated ILD, immunosuppression and anti-fibrotic drug regimens are relevant aspects mentioned.

Interstitial pneumonia with autoimmune features (IPAF) was defined for research purposes as interstitial lung disease (ILD) associated with features of autoimmunity without diagnosed rheumatic disease (RD). Since publication of the IPAF criteria in 2015, there have been multiple studies of IPAF. However, much remains unknown regarding pathogenesis, prognosis, and treatment in IPAF. This narrative review details the history and classification of IPAF, lists challenges associated with classifying patients as IPAF, and explores the prevalence, epidemiology, and presentation of IPAF. We also examine prognosis and important features determining IPAF clinical course, outline pathogenesis, and review treatment strategies.