Connective Tissue Disease-associated Interstitial Lung Disease: A review

Interstitial Lung Disease Program, Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, California.
Current respiratory care reports 09/2012; 1(4):224-232. DOI: 10.1007/s13665-012-0028-7
Source: PubMed


Interstitial lung disease (ILD) is commonly encountered in patients with connective tissue diseases (CTD). Besides the lung parenchyma, the airways, pulmonary vasculature and structures of the chest wall may all be involved, depending on the type of CTD. As a result of this so-called multi-compartment involvement, airflow limitation, pulmonary hypertension, vasculitis and extrapulmonary restriction can occur alongside fibro-inflammatory parenchymal abnormalities in CTD. Rheumatoid arthritis (RA), systemic sclerosis (SSc), poly-/dermatomyositis (PM/DM), Sjögren's syndrome (SjS), systemic lupus erythematosus (SLE), and undifferentiated (UCTD) as well as mixed connective tissue disease (MCTD) can all be associated with the development of ILD. Non-specific interstitial pneumonia (NSIP) is the most commonly observed histopathological pattern in CTD-ILD, but other patterns including usual interstitial pneumonia (UIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and lymphocytic interstitial pneumonia (LIP) may occur. Although the majority of patients with CTD-ILD experience stable or slowly advancing ILD, a small yet significant group exhibits a more severe and progressive course. Randomized placebo-controlled trials evaluating the efficacy of immunomodulatory treatments have been conducted only in SSc-associated ILD. However, clinical experience suggests that a handful of immunosuppressive medications are potentially effective in a sizeable portion of patients with ILD caused by other CTDs. In this manuscript, we review the clinical characteristics and management of the most common CTD-ILDs.

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Available from: Jeff Swigris, Dec 29, 2013
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    • "ИПЛ с картиной ОИП имеет более тяжелое течение, чем ИПЛ с другими гистологическими вариантами[31,33]. Преобладание модели ОИП отличает ИПЛ при РА от большинства других системных заболеваний соединительной ткани, как правило, характеризующихся преобладанием картины НИП[32,34]. Хотя РА чаще встречается у женщин, ИПЛ чаще выявляется у мужчин[9,10,19,31], соотношение мужчин и женщин – 2:1[10]. "

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    • "Several pathologic patterns of ILD are found in myositis, and as with other causes of ILD, the pathologic appearance impacts on prognosis regardless of the underlying aetiology: non-specific interstitial pneumonia (NSIP) is associated with a better prognosis than other subtypes. Connective tissue disease-associated ILD, including histopathologic subtypes, has been recently reviewed [24]. Diagnosis on CT chest patterns in patients with anti-Jo1 antibodies and anti-synthetase syndrome showed a preponderance of NSIP affecting 50% and usual interstitial pneumonia (UIP) affecting 30%. "
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