Elsevier

Clinics in Chest Medicine

Volume 25, Issue 3, September 2004, Pages 549-559
Clinics in Chest Medicine

Interstitial lung disease in the patient who has connective tissue disease

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Review of systems

Systemic complaints are common in many diseases; a detailed history and review of systems is critical in obtaining the correct ILD diagnosis. Box 1 is a checklist of symptoms and physical examination findings that commonly are associated with an underlying connective tissue disease. Some office practices have interstitial lung disease questionnaires so that errors of omission in symptom review do not occur.

Physical examination

Physical examination findings provide important clues to diagnosis of an underlying rheumatologic condition. A careful skin examination is an essential part of the physical examination. Skin findings in early systemic sclerosis (SSc) or scleroderma may be subtle; finger swelling (puffy fingers) often is the first specific SSc manifestation. This edematous phase is followed by sclerodactyly and thickening (sclerosis) of the skin more proximally. The term “limited cutaneous SSc” refers to skin

Laboratory screening

Laboratory screening commonly is used to decide if further CTD evaluation is needed. Laboratory tests that are used for screening purposes are neither sensitive nor perfectly specific for the CTD. A Westergren sedimentation rate is elevated in many infectious, malignant, and systemic diseases. Therefore, a high value is of limited benefit. Conversely, a low value may help to exclude active CTD, although normal values can be found occasionally.

Antinuclear antibodies (ANA) are found in most

Systemic lupus erythematosus

The American College of Rheumatology (ACR) publishes and updates the criteria that are necessary to establish a diagnosis of SLE. The most common pulmonary manifestations of disease are listed in Box 2. Patients who have evolving lupus may present with pulmonary manifestations without meeting formal criteria for the diagnosis. Patients who have SLE and diffuse lung disease require bronchoscopy to rule out infection or alveolar hemorrhage. Although infection is the most common cause of diffuse

Rheumatoid arthritis

RA is characterized by a symmetrical inflammatory arthritis and has a high prevalence of extra-articular manifestations. RA-associated pulmonary abnormalities are listed in Box 4. Although ILD is common in autopsy series, the frequency of clinically-significant ILD seems to be declining because disease-modifying antirheumatologic drugs are used with higher frequency. In the recent Spanish Registry of RA, ILD had a prevalence of 3.7% when diagnosed by chest radiography [12], [19]. Most patients

Systemic sclerosis

SSc (scleroderma) is a systemic fibrotic disease of unknown etiology that presents with two clinical phenotypes: ILD occurs with the limited (CREST variant, lcSSc) and diffuse (dcSSc) variants and is now a leading cause of morbidity and mortality (Box 5). Usually, ANA is positive in both subsets of disease: anticentromere or antibodies to T0/TH are predominant in limited disease [32] and an anti-SCl-70 antibody that is present on ENA panel is found more commonly in diffuse disease and is

Polymyositis/dermatomyositis

PM is a rare muscle disease that is characterized by proximal muscle weakness. Skin lesions that consist of violaceous scaling patches, Gottron's papules, facial heliotrope rash, or mechanic's hands characterize DM. Some patients who have DM without muscle disease (amyotrophic DM) also can have lung disease [44], [45]. The presentation of PM/DM diffuse lung disease was present in 65% of new diagnoses in one recent clinical series and was clinically silent in some individuals [46]. Lung disease

Sjögren's syndrome

SS is an autoimmune exocrinopathy and an autoimmune epithelitis that is characterized by lymphoproliferation and lymphocytic infiltration of glandular and nonglandular tissue. Typically, patients present with dry mouth (xerostomia), dry eyes (keratoconjunctivitis), and arthritis. SS is a complicating factor of many CTDs (secondary Sjögren's syndrome), but also may present without other CTDs (primary Sjögren's syndrome). Pulmonary symptoms of SS include cough and dyspnea. Usually, cough is from

Mixed connective tissue disease

MCTD is an overlap syndrome that combines features of SLE, SSc, RA, and PM/DM, that is combined with the presence of ribonucleoprotein (RNP) antibodies on ENA testing. Patients who have MCTD do not meet specific criteria for another CTD, but long-term follow-up of patients showed that most developed one of the more recognized CTD entities—usually scleroderma—over the next 5 years. Undifferentiated CTD differs from MCTD because MCTD requires the presence of an antibody to RNP. If the anti-RNP is

Undifferentiated connective tissue disease

Occasionally, patients present with individual features of a CTD but fail to meet ACR entry criteria for any single disease. These patients are classified as having undifferentiated connective tissue disease (UCTD). These individuals, like those who have MCTD, usually transition to other disease entities over time [63].

There are no studies that describe adequately the appropriate treatment of ILD in this population; most experts treat according to recommendations for the CTD that is most

Cryptogenic organizing pneumonia in the connective tissue diseases

Many of the CTDs have a bronchiolar and alveolar injury pattern on biopsy that heals with a loose connective tissue matrix that fills airways and airspaces (Fig. 2). Bronchiolar manifestations are more commonly seen in RA and SS; however, any of the CTDs can have an associated bronchiolitis. An organizing pneumonia that histologically contains loose connective tissue without recognizable infection also is seen in these same diseases with bronchiolar injury. The American Thoracic

Summary

Although rheumatologists often assume primary care for patients who have CTDs, their training in lung disease physiology, determining lung disease stability, and treatment of complications of ILDs, such as bronchiectasis, hypoxemia, pulmonary hypertension, and cough, require the assistance of a pulmonary physician for optimal management. Therefore, the successful and optimal management of these complicated patients is best achieved by a collaborative approach between the pulmonary physician and

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References (63)

  • C. Strange et al.

    Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension

    Chest

    (2000)
  • R.D. Sontheimer et al.

    Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis

    J Am Acad Dermatol

    (2003)
  • R.D. Sontheimer

    Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects

    Dermatol Clin

    (2002)
  • H. Arakawa et al.

    Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation

    Chest

    (2003)
  • O. Bonnefoy et al.

    Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis

    Eur J Radiol

    (2004)
  • A. Schnabel et al.

    Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment

    Semin Arthritis Rheum

    (2003)
  • J.H. Ryu et al.

    Cystic and cavitary lung diseases: focal and diffuse

    Mayo Clin Proc

    (2003)
  • M. Lippmann et al.

    Circulating antinuclear and rheumatoid factors in coal miners. A prevalence study in Pennsylvania and West Virginia

    Ann Intern Med

    (1973)
  • D.E. Aguilar Leon et al.

    Anti-avian antibodies and rheumatoid factor in pigeon hypersensitivity pneumonitis

    Clin Exp Allergy

    (2003)
  • D.M. Lee et al.

    Clinical utility of the anti-CCP assay in patients with rheumatic diseases

    Ann Rheum Dis

    (2003)
  • D. Williams

    Rheumatoid arthritis: autoimmunity in rheumatoid arthritis

  • M. Ishii et al.

    Possible association of “shrinking lung” and anti-Ro/SSA antibody

    Arthritis Rheum

    (2000)
  • C.M. Laroche et al.

    Diaphragm strength in the shrinking lung syndrome of systemic lupus erythematosus

    QJM

    (1989)
  • E. Tanaka et al.

    Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment

    J Rheumatol

    (2002)
  • Y. Nanke et al.

    [Isolated pulmonary hypertension in overlap syndrome: successful treatment by methylprednisolone pulse therapy]

    Nihon Rinsho Meneki Gakkai Kaishi

    (2003)
  • M.R. Zamora et al.

    Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome

    Medicine (Baltimore)

    (1997)
  • C.K. Lee et al.

    Pulmonary alveolar hemorrhage in patients with rheumatic diseases in Korea

    Scand J Rheumatol

    (2000)
  • R.D. Brasington et al.

    Pulmonary disease in systemic lupus erythematosus

    Clin Exp Rheumatol

    (1985)
  • G.S. Cheema et al.

    Interstitial lung disease in systemic lupus erythematosus

    Curr Opin Pulm Med

    (2000)
  • L. Carmona et al.

    Rheumatoid arthritis in Spain: occurrence of extra-articular manifestations and estimates of disease severity

    Ann Rheum Dis

    (2003)
  • M.J. Soler et al.

    Fatal, gold-induced pneumonitis

    Rheumatol Int

    (2003)

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      We believe that in IPAF patients, a validated questionare to detect xerophtalmia should be done, in order to increase the suspicion of SS and, therefore perform minimally invasive procedures, such as MSGB and/or DET to confirm SS. Approximately 15–20% of patients presenting with ILD have an occult connective tissue disease [19]. Lung involvement can occur before usual manifestations of SS [20,21].

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