Pulmonary Pathology of the Rheumatic Diseases

Abstract

Thoracic manifestations of the rheumatic diseases (RDs) are a significant cause of morbidity and mortality worldwide. The five RDs most frequently associated with pleuropulmonary disease are (1) rheumatoid arthritis (RA), (2) systemic lupus erythematosus (SLE), (3) progressive systemic sclerosis (PSS), (4) polymyositis/dermatomyositis (PM/DM), and (5) Sjögren syndrome (SS). The clinical presentation is highly variable, ranging from pleuritic pain alone to slowly progressive breathlessness accompanied by cough. On occasion RD may present acutely with overwhelming respiratory failure as the first pulmonary manifestation. In all of the RDs, the pathology is dominated by either or both inflammation and fibrosis, but the anatomical distribution in each varies somewhat. For example, airway-associated lymphoid hyperplasia is a common manifestation of SS, PM/DM preferentially involves the alveolar parenchyma, and pleural inflammation is most commonly seen in RA. Such changes may be detected radiologically as peribronchovascular disease in SS, ground-glass infiltrates in PM/DM, and pleural disease with effusion in RA. Some RDs are more commonly associated with distinctive histopathology, such as rheumatoid nodules in RA, or lymphoid hyperplasia in both RA and SS. Patients with PSS are at particular risk for pulmonary hypertension when the lung is involved. Also, statistically, acute lung injury occurs more commonly in some, such as SLE and PM/DM. The very common use of immunosuppressive agents in the treatment of RD creates additional diagnostic problems related to drug toxicity and infection. Finally, a major confounding factor occurs when preclinical RD presents first as pulmonary disease (particularly PM/DM), an occurrence that may invoke one of the so-called idiopathic interstitial pneumonias. The most common pleuropulmonary pathological manifestations of the five major RDs are presented and discussed with brief radiological correlations.

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

“…The evaluation of lung parenchyma obtained by surgical lung biopsy may provide clues about whether an underlying CTD is present [50,51]. The histopathologic features included within the morphologic domain criteria for IPAF are only those considered to be highly associated with, but not diagnostic for, the presence of CTD [50,51].…”

“…The histopathologic features included within the morphologic domain criteria for IPAF are only those considered to be highly associated with, but not diagnostic for, the presence of CTD [50,51]. These are the primary patterns of NSIP, OP and LIP and the secondary features of interstitial lymphoid aggregates with germinal centres and diffuse lymphoplasmacytic infiltration with or without lymphoid follicles.…”

“…In addition to interstitial pneumonia, the presence of several concurrent thoracic compartment manifestations is another characteristic often encountered among patients with CTD [45,46,51]. In this section of the morphologic domain, we consider "multi-compartment involvement", which includes unexplained airways, vascular, pleural or pericardial abnormalities.…”

Padrões tomográficos das doenças intersticiais pulmonares difusas com correlação clínica e patológica

“…(70) Hypersensitivity pneumonitis Centrilobular nodules of ground-glass opacity distributed diffusely and profusely through the Connective tissue disease-related interstitial lung disease Like drugs, systemic autoimmune diseases can produce a wide variety of histopathological patterns. (76) In patients previously diagnosed with connective tissue disease (CTD), DAD or acute OP can occur-or the pneumonitis can represent the initial manifestation of disease, especially in systemic lupus erythematosus, polymyositis, and adult-onset Still's disease. (77,78) Serum creatine phosphokinase and ferritin should be measured in order to evaluate the last two.…”

Influenza Vaccination in Autoimmune Rheumatic Disease Patients

“…Infections are one of the leading causes of mortality in these patients (Doran et al 2002;Cervera et al 2003;Falagas et al 2007). Pulmonary infections are a significant cause of morbidity and mortality in autoimmune rheumatic diseases (Leslie et al 2007). Viral and bacterial infections in patients suffering from autoimmune rheumatic diseases can have a dramatic course due to the use of aggressive immunosuppressive therapy protocols.…”