Clinical Course, Prognosis, and Causes of Death in Mixed Connective Tissue Disease

Hajas, A.; Szodoray, Péter; Nakken, Britt; Gaál, János; Zöld, Éva; Laczik, Renáta; Demeter, Nora; Nagy, Gábor; Szekanecz, Zoltán; Zeher, Margit; Szegedi, Gyula; Bodolay, Edit

doi:10.3899/jrheum.121272

Cited by 100 publications

(83 citation statements)

References 47 publications

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“…This might suggest that subclinical ILD is common, and a more proactive screening strategy should be employed in clinical practice. We also confirmed the rarity of CNS and renal involvement, similar to the original description by Sharp et al .…”

Section: Discussionsupporting

confidence: 91%

Epidemiology of Mixed Connective Tissue Disease, 1985–2014: A Population‐Based Study

Ungprasert

Crowson

Chowdhary

et al. 2016

Arthritis Care & Research

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Objectives To characterize the epidemiology of mixed connective tissue disease (MCTD) from 1983 to 2014. Methods An inception cohort of patients with incident MCTD in 1985-2014 in Olmsted County, Minnesota was identified based on comprehensive individual medical record review. Diagnosis of MCTD required fulfillment of at least one of the four widely-accepted diagnostic criteria without fulfillment of classification criteria for other connective tissue diseases. Data were collected on demographic characteristics, clinical presentation, laboratory investigations and mortality. Results A total of 50 incident cases of MCTD were identified (mean age 48.1 years and 84% were female). The annual incidence of MCTD was 1.9 per 100,000 population. Raynaud's phenomenon was the most common initial symptoms (50%) followed by arthralgia (30%) and swollen hands (16%). The diagnosis was frequently delayed with the median time from first symptom to fulfillment of criteria of 3.6 years. At fulfillment of criteria, arthralgia was the most prevalent manifestation (86%) followed by Raynaud's phenomenon (80%), swollen hands (64%), leukopenia/lymphopenia (44%) and heartburn (38%). Evolution to other connective tissue occurred infrequently with 10-year rate of evolution of 8.5% and 6.3% for SLE and SSc, respectively. The overall mortality was not different form the general population with standardized mortality ratio: 1.1 (95% CI, 0.4-2.6). Conclusions This study was the first population-based study of MCTD to provide a complete picture of epidemiology and clinical characteristics of MCTD. MCTD occurred in about 2 persons per 100,000 per year. Evolution to other connective diseases occurred infrequently and the mortality was not affected.

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Section: Discussionsupporting

confidence: 91%

Epidemiology of Mixed Connective Tissue Disease, 1985–2014: A Population‐Based Study

Ungprasert

Crowson

Chowdhary

et al. 2016

Arthritis Care & Research

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“…PH is increased in prevalence in CTDs, especially in SSc, SLE, MCTD and to a lesser extent in RA, SS and PM/DM [63,64,65,66]. PH is a malignant prognostic determinant, and for this reason alone, irrespective of treatment considerations, the identification of PH is an important part of routine monitoring.…”

Section: Ph In Monitoringmentioning

confidence: 99%

Monitoring of Lung Involvement in Rheumatologic Disease

Paschalaki

Jacob²,

Wells

2016

Respiration

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The monitoring of lung involvement in patients with connective tissue diseases is central to optimal long-term management and is directed towards: (a) the detection of supervening lung involvement not present at presentation and (b) the identification of disease progression in established lung disease. For both goals, accurate surveillance requires multi-disciplinary evaluation with the integration of symptomatic change, serial pulmonary function trends and imaging data. Evaluated in isolation, each of these monitoring domains has significant limitations. Symptomatic change may be confounded by a wide variety of systemic factors. Pulmonary function tests provide the most reliable data, but are limited by measurement variability, the heterogeneity of functional patterns and the confounding effects of non-pulmonary factors. Chest radiography is insensitive to change but may provide rapid confirmation of major disease progression or alert the clinician to respiratory co-morbidities. Although high-resolution computed tomography has a central role in assessing disease severity, it should be used very selectively as a monitoring tool due to the associated radiation burden. Ancillary tests include echocardiography and exercise testing to proactively identify cases of pulmonary hypertension and worsening of oxygenation. In summary, a multi-disciplinary approach is essential for the identification of disease progression and prompt treatment of comorbidities that severely impact on the morbidity and mortality of disease.

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“…Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in the ambit of almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure - in most cases requiring ventilatory support - as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event [1,2,3,4,5,6,7,8,9,10]. …”

Section: Introductionmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the ‘mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the ‘vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders.

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Clinical Course, Prognosis, and Causes of Death in Mixed Connective Tissue Disease

Abstract: Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.

Cited by 100 publications

References 47 publications

Epidemiology of Mixed Connective Tissue Disease, 1985–2014: A Population‐Based Study

Epidemiology of Mixed Connective Tissue Disease, 1985–2014: A Population‐Based Study

Monitoring of Lung Involvement in Rheumatologic Disease

Acute Respiratory Events in Connective Tissue Disorders

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