Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Interstitial Lung Disease Associated With Collagen-Vascular Disease Workup

  • Author: Isabel F Pedraza, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
 
Updated: Sep 20, 2013
 

Laboratory Studies

Laboratory studies are helpful in the setting of collagen-vascular disease (CVD) associated with interstitial lung disease (ILD). At least 15% of patients with ILD have evidence of underlying CVD.[2] Various antibodies detected in the serum of patients with CVD help in determining the diagnosis and the prognosis (see Table 2 below).

Table 2. Autoantibodies in Collagen-Vascular Diseases (Open Table in a new window)

Autoantibody RA SLE SD SS PM/DM AS MCTD
RF + + + + Rare - +
ANA + + + + Rare - +



(speckled)



ds-DNA - + - - - - -
Anticentromere - - + (limited) Rare Rare - -
Scl-70 - - + (diffuse)   Rare - -
Anti-Jo - - - Rare + (ILD) - -
ANCA Rare Rare - - - - -
Smith antibody - + - - - - -
Anti-Ro/SSA and anti-La/SSB - - - + - - -
Anti-U1-RNP and anti-UN-70 kd - - - - - - +
Anti-CCP + - - - - - -
ANA = antinuclear antibody; ANCA = antineutrophilic cytoplasmic antibody; AS = ankylosing spondylitis; DM = dermatomyositis; ds-DNA = double-stranded DNA antibody; ILD = interstitial lung disease; MCTD = mixed connective-tissue disease; PM = polymyositis; RA = rheumatoid arthritis; RF = rheumatoid factor; RNP = ribonucleoprotein; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; CCP = cyclic citrullinated peptide.

Anemia of chronic disease can be found in persons with rheumatoid arthritis (RA), whereas systemic lupus erythematosus (SLE) can cause leukopenia, lymphopenia, thrombosis, and thrombocytopenia. The erythrocyte sedimentation rate (ESR) and creatine kinase levels may be high in patients with polymyositis (PM)/dermatomyositis (DM). Total complement levels and a high ESR may be present in SLE patients who present with an acute lupus flare.

Next

Plain Radiography and Computed Tomography

Radiologically, CVDs may manifest as a focal or a diffuse pulmonary abnormality. The type and frequency of the lung abnormalities vary with the specific disease (see Table 2 below).

Table 3. Radiographic Patterns of Collagen-Vascular Diseases (Open Table in a new window)

Radiologic Pattern RA SLE SD Secondary SS PM/DM AS MCTD
Pleural effusion + + + ± - - +
Interstitial pneumonitis, fibrosis UIP and NSIP patterns + LIP pattern ± + Upper apical fibrosis[46] +
BOOP ± + + + + ± ±
Pulmonary nodules Rheumatoid pulmonary nodules; uncommon, may be 1-5 mm, single or multiple, may cavitate - - Follicular lymphoid hyperplasia or lymphoma can present as lung nodules + - -
Bronchiectasis + + + + + + +
Caplan syndrome Coal worker’s pneumoconiosis, rheumatoid nodules - - - - - ±
Diffuse pulmonary hemorrhage - + - - - - ±
Shrinking lung syndrome - Loss of lung volume at bases with no parenchymal pathology - - - - ±
Diaphragmatic dysfunction - + - May be present + - ±
Cysts, honeycombing 10% of patients have subpleural honeycombing; compared with IPF, it is more anterior and involves upper lobes Uncommon + Present, especially in LIP + Upper-lobe cyst may become infected with Aspergillus species -
GGO Present, especially in NSIP + + + + - ±
AS = ankylosing spondylitis; BOOP = bronchiolitis obliterans organizing pneumonia; DM = dermatomyositis; GGO = ground-glass opacification; IPF = idiopathic pulmonary fibrosis; LIP = lymphoid interstitial pneumonia; MCTD = mixed connective-tissue disease; NSIP = nonspecific interstitial pneumonia; PM = polymyositis; RA = rheumatoid arthritis; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; UIP = usual interstitial pneumonia.

RA, SLE, scleroderma (SD), Sjögren syndrome (SS), and PM/DM all can cause an interstitial fibrosis similar to interstitial pulmonary fibrosis (IPF). All CVDs like IPF involve lung bases and are subpleural, except for ankylosing spondylitis (AS), which involves the upper lobes, and RA-induced ILD. RA-ILD is differentiated from IPF on the basis of greater upper-lobe involvement, an anterior location, and the presence of reticular infiltrates finer than those associated with IPF (see the image below).

High-resolution CT scan of advanced-stage pulmonar High-resolution CT scan of advanced-stage pulmonary fibrosis demonstrating reticular opacities with honeycombing in predominantly subpleural distribution. This pattern can be present in rheumatoid arthritis–related interstitial lung disease, Sjögren syndrome, and scleroderma.

Initially, chest radiographs may be normal[47] ; however, in later stages of the disease, they may show fine reticulation. High-resolution computed tomography (HRCT) is more sensitive for evaluation of CVD-related ILD. Depending on the type of CVD present, radiography may reveal pleural effusion, nodules, bronchiectasis, loss of lung volume, or prominent pulmonary vessels. Ground-glass opacities and reticulation are common findings.

Numerous studies show correlation HRCT findings and pulmonary function test (PFT) results correlate with underlying lung histopathology in patients with CVD.[48] Ground-glass opacities (GGOs) and consolidation may reflect the presence of interstitial pneumonia on computed tomography (CT) of the chest (see the image below). Pulmonary fibrosis is uncommon in SLE patients; if it occurs, it usually is patchy. The abnormalities occur mainly at the periphery of the lung and can be associated with traction bronchiectasis and honeycombing. The HRCT findings in CVD-associated ILD can be indistinguishable from those found in the idiopathic interstitial pneumonias.

Ground-glass opacification (GGO) may correlate wit Ground-glass opacification (GGO) may correlate with active alveolitis and favorable response to therapy. GGO is among earliest features of rheumatoid arthritis–induced interstitial lung disease.

RA is associated with the following 4 CT patterns:

  • Usual interstitial pneumonia (UIP)
  • Nonspecific interstitial pneumonia (NSIP)
  • Bronchiolitis [49]
  • Organizing pneumonia [50]

The most common CT features of RA-related lung disease are GGOs and reticulation.[12, 51] RA can manifest as rheumatoid nodules, Caplan syndrome (rheumatoid nodules up to 5 cm, mostly involving the upper lungs in coal miners and resembling coal worker’s pneumoconiosis).[52] In a smoker who has RA and presents with lung nodules, lung cancer should be ruled out first.[53]

In patients with SD, HRCT scanning frequently shows evidence of interstitial pneumonitis and fibrosis, mainly involving the lower lobes, in a predominantly peripheral and posterior distribution.

PM/DM-induced lung disease is rare (5%). The most common pattern of ILD is symmetric and predominantly basal reticulation. HRCT scanning is remarkable for revealing prominent interlobular septa, patchy consolidation, and honeycombing. Patients with an acute presentation have GGOs and consolidation, in contrast to the reticulation and honeycombing seen in patients with the chronic type of ILD.

SS is manifested by a reticulonodular pattern of infiltrates involving lower lung zones. This finding may reflect the presence of lymphocytic interstitial fibrosis (see the image below). HRCT scans may reveal GGOs and bronchiolitis obliterans.[54]

Patient with lymphocytic interstitial pneumonia. Patient with lymphocytic interstitial pneumonia.

Radiologic features of MCTD vary across different studies. Radiologic abnormalities include subpleural honeycombing, BOOP, and pleural effusion.

Previous
Next

Pulmonary Function Tests

PFTs include spirometry, lung volumes, diffusion capacity of the lung for carbon monoxide (DLCO), and arterial blood gas (ABG) measurements.

Most CVDs cause a restrictive lung disease pattern with a decrease in total lung capacity (TLC), residual volume (RV), functional residual capacity, and DLCO.[55] Forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) (ie, the FEV1/FVC ratio) may be normal or increased. However, bronchiolitis obliterans may cause an obstructive ventilatory defect (reduced FEV1/FVC ratio and FEV1, increased RV and RV/TLC ratio).

ABG analysis may reveal hypoxemia at rest. Arterial oxygen desaturation may occur with exercise. A 6-minute walk test with pulse oximetry provides a measure of oxygen desaturation and helps to detect disease progression.

Previous
Next

Other Tests

Schirmer and Rose Bengal stain

The Schirmer test may be used to screen for dry eyes secondary to decreased tear production in patients with SS. Similarly, Rose Bengal staining of the cornea can detect keratitis associated with SS.

Echocardiography

Echocardiography can help detect cardiac involvement, which is useful in patients presenting with heart failure and in patients with suspected pulmonary hypertension.

Gallium scanning

Gallium scanning results may be abnormal in patients with CVDs, probably as a consequence of alveolitis. However, the role of gallium scanning in the diagnosis or prognosis of CVDs is not well established.

Previous
Next

Bronchoalveolar Lavage

Bronchoalveolar lavage (BAL) results are not diagnostic in patients with CVDs, but they are helpful in determining the prognosis and guiding treatment.

Studies have been performed to determine the importance of cell counts in BAL samples; patients with increased neutrophil counts tend to have worse prognoses than those with increased lymphocyte counts.[56] BAL results seem to correlate with the underlying lung pathology.[57, 58] Similarly, BAL lymphocytosis may predict corticosteroid responsiveness. Most patients with underlying ILD have lymphocytosis in BAL fluid. Finally, BAL is valuable for excluding infections that can mimic CVDs.

Previous
Next

Biopsy

As with other ILDs, a transbronchial biopsy is usually inadequate for diagnosis, given the often patchy distribution of histologic findings, and, often, open lung biopsy is required.

HRCT results correlate well with the pathology of the underlying CVD.[59] However, open lung biopsy is needed in atypical cases,[60] depending on the clinical and functional status of the patient. Video-assisted thoracoscopic surgery is usually preferred.

Previous
Next

Histologic Findings

The most common histopathologic findings in patients with CVDs are UIP, NSIP, BOOP, follicular bronchiolitis, diffuse alveolar hemorrhage (DAH), and lymphoid interstitial pneumonia (LIP).

Usual interstitial pneumonia

Histologic findings in UIP include fibroblastic foci with alternate areas of normal lung tissue, fibrosis, and honeycombing (see the image below). Distribution is peripheral, subpleural, and basal. These findings may be seen in SLE, RA, DM/PM, and MCTD.

Usual interstitial pneumonitis. Subpleural and par Usual interstitial pneumonitis. Subpleural and paraseptal inflammation are present, with appearance of temporal heterogeneity. Patchy scarring of lung parenchyma and normal (or nearly normal) alveoli interspersed between fibrotic areas are hallmarks of this disease. In addition, lung architecture is completely destroyed. This pattern can be present in rheumatoid arthritis–induced interstitial lung disease and generally is associated with poor prognosis.

Nonspecific interstitial pneumonia

Histologic findings in NSIP[61] include varying proportions of interstitial inflammation and fibrosis, which may be divided into cellular and fibrosing and are patchy with intervening normal lung tissue. Distribution is peripheral, subpleural, basal, and symmetric. These findings may be seen in SLE, RA, DM/PM, and MCTD.

Lymphoid interstitial pneumonia

Histologic findings in LIP include infiltration of T cells, plasma cells, and macrophages, as well as lymphoid hyperplasia that is usually diffuse and predominantly septal. Distribution is diffuse. These findings may be seen in SS and MCTD.

Bronchiolitis obliterans organizing pneumonia

Histologic findings in BOOP include patchy intraluminal organizing fibrosis in air spaces with preservation of lung architecture. Distribution is bronchovascular. These findings may be seen in SLE, SD, SS, and PM/DM.

Diffuse alveolar hemorrhage

Histologic findings in DAH include hyaline membrane formation, damaged type II pneumocytes, alveolar edema, and fibroblastic proliferation. Distribution is diffuse. These findings may be seen in SLE, RA, SD, and PM/DM.

Previous
 
 
Contributor Information and Disclosures
Author

Isabel F Pedraza, MD Director, Respiratory Intensive Care Unit, Faculty Physician, Department of Medicine, Division of Pulmonary/Critical Care Medicine, Women's Guild Lung Institute, Cedars-Sinai Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, Society of Critical Care Medicine, American Thoracic Society

Disclosure: Nothing to disclose.

Arshad Ali, MD Attending Physician, Department of Pulmonary and Critical Care Medicine, Mercy General Hospital of Sacramento

Arshad Ali, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Danilo A Enriquez, MD, FCCP Clinical Assistant Professor of Medicine, State University of New York Health Science Center at Brooklyn; Associate Program Director of Internal Medicine Residency Program, Interfaith Medical Center

Danilo A Enriquez, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association

Disclosure: Nothing to disclose.

Hina Arif, MD 

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H Professor, Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California Keck School of Medicine

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Osler Society, American Thoracic Society, New York Academy of Medicine, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. Self SE. Autoantibody testing for autoimmune disease. Clin Chest Med. 2010 Sep. 31(3):415-22. [Medline].

  2. Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. 2004 Sep. 25(3):549-59, vii. [Medline].

  3. Tzelepis GE, Toya SP, Moutsopoulos HM. Occult connective tissue diseases mimicking idiopathic interstitial pneumonias. Eur Respir J. 2008 Jan. 31(1):11-20. [Medline].

  4. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010 Aug. 138(2):251-6. [Medline].

  5. Castagnaro A, Chetta A, Marangio E, Zompatori M, Olivieri D. The lung in immune-mediated disorder: rheumatoid arthritis. Curr Drug Targets Inflamm Allergy. Dec; 2004. 3(4):449-54. [Medline].

  6. Olson AL, Swigris JJ. Idiopathic pulmonary fibrosis: diagnosis and epidemiology. Clin Chest Med. 2012 Mar. 33(1):41-50. [Medline].

  7. Jindal SK, Agarwal R. Autoimmunity and interstitial lung disease. Curr Opin Pulm Med. Sep;2005. 11(5):438-46. [Medline].

  8. Antin-Ozerkis D, Rubinowitz A, Evans J, Homer RJ, Matthay RA. Interstitial lung disease in the connective tissue diseases. Clin Chest Med. 2012 Mar. 33(1):123-49. [Medline].

  9. Meyer KC, Decker C, Baughman R. Toxicity and monitoring of immunosuppressive therapy used in systemic autoimmune diseases. Clin Chest Med. 2010 Sep. 31(3):565-88. [Medline].

  10. Wells AU, Steen V, Valentini G. Pulmonary complications: one of the most challenging complications of systemic sclerosis. Rheumatology (Oxford). 2009 Jun. 48 Suppl 3:iii40-4. [Medline].

  11. Kamen DL, Strange C. Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med. 2010 Sep. 31(3):479-88. [Medline].

  12. Tanaka N, Kim JS, Newell JD, Brown KK, Cool CD, Meehan R, et al. Rheumatoid arthritis-related lung diseases: CT findings. Radiology. Jul;2004 Epub 2004 May 27. 232(1):81-91. [Medline].

  13. Antin-Ozerkis D, Evans J, Rubinowitz A, Homer RJ, Matthay RA. Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med. 2010 Sep. 31(3):451-78. [Medline].

  14. Kalluri M, Oddis CV. Pulmonary manifestations of the idiopathic inflammatory myopathies. Clin Chest Med. 2010 Sep. 31(3):501-12. [Medline].

  15. Khan MA, Mathieu A, Sorrentino R, Akkoc N. The pathogenetic role of HLA-B27 and its subtypes. Autoimmun Rev. Jan;2007 Epub 2006 Dec. 6(3):183-9. [Medline].

  16. Cooper GS, Dooley MA, Treadwell EL, St Clair EW, Parks CG, Gilkeson GS. Hormonal, environmental, and infectious risk factors for developing systemic lupus erythematosus. Arthritis Rheum. Oct;1998. 41(10):1714-24. [Medline].

  17. Saag KG, Kolluri S, Koehnke RK, Georgou TA, Rachow JW, Hunninghake GW, et al. Rheumatoid arthritis lung disease. Determinants of radiographic and physiologic abnormalities. Arthritis Rheum. Oct;1996. 39(10):1711-9. [Medline].

  18. Hagaman JT, Panos RJ, McCormack FX, et al. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung diseases. Chest. 2011 Feb. 139(2):353-60. [Medline]. [Full Text].

  19. Ruiz-Irastorza G, Khamashta MA, Castellino G, Hughes GR. Systemic lupus erythematosus. Lancet. Mar 31;2001. 357(9261):1027-32. [Medline].

  20. Tamaki T, Mori S, Takehara K. Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res. 1991;. 283(6):366-71. [Medline].

  21. Maricq HR, Weinrich MC, Keil JE, Smith EA, Harper FE, Nussbaum AI, et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum. Aug;. 32(8):1989:998-1006. [Medline].

  22. Kurland LT, Hauser WA, Ferguson RH, Holley KE. Epidemiologic features of diffuse connective tissue disorders in Rochester, Minn., 1951 through 1967, with special reference to systemic lupus erythematosus. Mayo Clin Proc. Sep;1969. 44(9):649-63. [Medline].

  23. Oddis CV, Conte CG, Steen VD, Medsger TA. Incidence of polymyositis-dermatomyositis: a 20-year study of hospital diagnosed cases in Allegheny County, PA 1963-1982. J Rheumatol. Oct;1990. 17(10):1329-34. [Medline].

  24. Spector TD. Rheumatoid arthritis. Rheum Dis Clin North Am. Aug;1990. 16(3):513-37. [Medline].

  25. Kellgren JH. Epidemiology of rheumatoid arthritis. Arthritis Rheum. Oct;1966. 9(5):658-74. [Medline].

  26. Youinou P, Moutsopoulos HM, Pennec YL. Clinical features of Sjögren's syndrome. Curr Opin Rheumatol. Oct;1990. 2(5):687-93. [Medline].

  27. Fessel WJ. Epidemiology of systemic lupus erythematosus. Rheum Dis Clin North Am. Apr;. 14(1): 1988:15-23. [Medline].

  28. Lawrence RC, Hochberg MC, Kelsey JL, McDuffie FC, Medsger TA, Felts WR, et al. Estimates of the prevalence of selected arthritic and musculoskeletal diseases in the United States. J Rheumatol. Apr;1989. 16(4):427-41. [Medline].

  29. Hagiwara K, Sato T, Takagi-Kobayashi S, Hasegawa S, Shigihara N, Akiyama O. Acute exacerbation of preexisting interstitial lung disease after administration of etanercept for rheumatoid arthritis. J Rheumatol. May;2007 Epub 2007 Apr 15. 34(5):1151-4. [Medline].

  30. Kim DS. Interstitial lung disease in rheumatoid arthritis: recent advances. Curr Opin Pulm Med. Sep;. 12(5):2006:346-53. [Medline].

  31. Mouthon L, Berezné A, Brauner M, Valeyre D, Guillevin L. Interstitial lung disease in connective tissue disorders. Rev Pneumol Clin. Jun;2005. 61(3):211-9. [Medline].

  32. Kocheril SV, Appleton BE, Somers EC, et al. Comparison of disease progression and mortality of connective tissue disease-related interstitial lung disease and idiopathic interstitial pneumonia. Arthritis Rheum. 2005 Aug 15. 53(4):549-57. [Medline].

  33. Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007 Apr 1. 175(7):705-11. [Medline].

  34. Takizawa H, Suzuki N, Yanagawa T, Okazaki H, Satoh M, Akiyama N, et al. Importance of interstitial lung disease in collagen vascular disease: analysis of outcome. Nihon Kyobu Shikkan Gakkai Zasshi. Nov;1996. 34(11):1177-81. [Medline].

  35. Hakala M. Poor prognosis in patients with rheumatoid arthritis hospitalized for interstitial lung fibrosis. Chest. Jan;1988. 93(1):114-8. [Medline].

  36. Kang EH, Lee EB, Shin KC, Im CH, Chung DH, Han SK, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford). Oct: 2005;Epub 2005 Jun 21. 44(10):1282-6. [Medline].

  37. Takizawa H, Suzuki N, Yanagawa T, Okazaki H, Satoh M, Akiyama N, et al. Pulmonary involvement of collagen vascular diseases: studies on prognostic factors from basic and clinical viewpoints. Nihon Kyobu Shikkan Gakkai Zasshi. Dec;1995. 33 Suppl:291-5. [Medline].

  38. Matthay RA, Schwarz MI, Petty TL, Stanford RE, Gupta RC, Sahn SA, et al. Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis. Medicine (Baltimore). Sep;1975. 54(5):397-409. [Medline].

  39. Martens J, Demedts M, Vanmeenen MT, Dequeker J. Respiratory muscle dysfunction in systemic lupus erythematosus. Chest. Aug;1983. 84(2):170-5. [Medline].

  40. Owens GR, Follansbee WP. Cardiopulmonary manifestations of systemic sclerosis. Chest. Jan;1987. 91(1):118-27. [Medline].

  41. Keane MP, Lynch JP. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax. Feb;2000. 55(2):159-66. [Medline].

  42. Onomura K, Nakata H, Tanaka Y, Tsuda T. Pulmonary hemorrhage in patients with systemic lupus erythematosus. J Thorac Imaging. Apr;1991. 6(2):57-61. [Medline].

  43. Won Huh J, Soon Kim D, Keun Lee C, Yoo B, Bum Seo J, Kitaichi M, et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis. Respir Med. Aug;2007:Epub 2007 Apr 10. 101(8):1761-9. [Medline].

  44. Wiener-Kronish JP, Solinger AM, Warnock ML, Churg A, Ordonez N, Golden JA. Severe pulmonary involvement in mixed connective tissue disease. Am Rev Respir Dis. Oct;1981. 124(4):499-503. [Medline].

  45. Végh J, Szilasi M, Soós G, Dévényi K, Dezso B, Soltész P, et al. Interstitial lung disease in mixed connective tissue disease. Orv Hetil. Nov 27;2005. 146(48):2435-43. [Medline].

  46. Fenlon HM, Casserly I, Sant SM, Breatnach E. Plain radiographs and thoracic high-resolution CT in patients with ankylosing spondylitis. AJR Am J Roentgenol. Apr;1997. 168(4):1067-72. [Medline].

  47. Gabbay E, Tarala R, Will R, Carroll G, Adler B, Cameron D, et al. Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med. Aug;1997. 156(2 Pt 1):528-35. [Medline].

  48. Cortet B, Perez T, Roux N, Flipo RM, Duquesnoy B, Delcambre B, et al. Pulmonary function tests and high resolution computed tomography of the lungs in patients with rheumatoid arthritis. Ann Rheum Dis. Oct;1997. 56(10):596-600. [Medline].

  49. Herzog CA, Miller RR, Hoidal JR. Bronchiolitis and rheumatoid arthritis. Am Rev Respir Dis. Nov;1981. 124(5):636-9. [Medline].

  50. Corrin B, Turner-Warwick M, Geddes DM, Brewerton DA. Bronchiolitis obliterans. A new form of rheumatoid lung?. Chest. Feb;1978. 73(2):244. [Medline].

  51. Cortet B, Flipo RM, Rémy-Jardin M, Coquerelle P, Duquesnoy B, Rêmy J, et al. Use of high resolution computed tomography of the lungs in patients with rheumatoid arthritis. Ann Rheum Dis. Oct;1995. 54(10):815-9. [Medline].

  52. CAPLAN A. Certain unusual radiological appearances in the chest of coal-miners suffering from rheumatoid arthritis. Thorax. Mar;1953. 8(1):29-37. [Medline].

  53. Jolles H, Moseley PL, Peterson MW. Nodular pulmonary opacities in patients with rheumatoid arthritis. A diagnostic dilemma. Chest. Nov;1989. 96(5):1022-5. [Medline].

  54. Franquet T, Giménez A, Monill JM, Díaz C, Geli C. Primary Sjögren's syndrome and associated lung disease: CT findings in 50 patients. AJR Am J Roentgenol. Sep;1997. 169(3):655-8. [Medline].

  55. Gabazza E, Taguchi O, Yamakami T, Machishi M, Ibata H, Suzuki S. Usefulness of DLco for the early diagnosis of pulmonary involvement in collagen diseases. Nihon Kyobu Shikkan Gakkai Zasshi. Apr;1993. 31(4):480-5. [Medline].

  56. Chhajed PN, Doshi KP, Athavale AU, Bichile LS, Shah AC. Bronchoalveolar lavage (BAL) in newly diagnosed patients with collagen vascular diseases. Indian J Chest Dis Allied Sci. Oct-Dec;1998. 40(4):243-50. [Medline].

  57. Popp W, Braun O, Ritschka L, Scherak O, Kolarz G, Rauscher H, et al. Chest X-ray in collagen vascular diseases. A comparison of chest X-ray with bronchoalveolar lavage and transbronchial forceps biopsy. Respiration. 1994;. 61(3):138-43. [Medline].

  58. Nagai S, Satake N, Kitaichi M, Izumi T. Interstitial pneumonia associated with collagen vascular diseases: histological findings, and cells in bronchoalveolar lavage fluid. Nihon Kyobu Shikkan Gakkai Zasshi. Dec;1995. 33 :Suppl:258-63. [Medline].

  59. Tanaka N, Newell JD, Brown KK, Cool CD, Lynch DA. Collagen vascular disease-related lung disease: high-resolution computed tomography findings based on the pathologic classification. J Comput Assist Tomogr. May-Jun; 2004. 28(3):351-60. [Medline].

  60. Grubben MJ, Kerstens PJ, Wiersma JM, Boerbooms AM, Festen J. Pleuro-pulmonary involvement in patients with connective tissue disease. The role of open lung biopsy. Neth J Med. Dec;1993. 43(5-6):269-76. [Medline].

  61. Sato T, Fujita J, Yamadori I, Ohtsuki Y, Yoshinouchi T, Bandoh S, et al. Non-specific interstitial pneumonia; as the first clinical presentation of various collagen vascular disorders. Rheumatol Int. Apr; 2006: Epub 2005 Nov 8. 26(6):551-5. [Medline].

  62. Kuwana M. Collagen diseases with pulmonary involvement. Nihon Rinsho Meneki Gakkai Kaishi. Jun; 2004. 27(3):118-26. [Medline].

  63. Fink SD, Kremer JM. Successful treatment of interstitial lung disease in systemic lupus erythematosus with methotrexate. J Rheumatol. May;1995. 22(5):967-9. [Medline].

 
Previous
Next
 
Pulmonary hypertension is complication of various collagen-vascular diseases. Lung biopsy specimen demonstrates severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of pulmonary arteriole, compatible with pulmonary hypertension.
Heliotrope rash in woman with dermatomyositis.
Gottron papules and nail-fold telangiectasia in patient with dermatomyositis.
Classic malar rash (butterfly rash) with distribution over cheeks and nasal bridge. Note that fixed erythema (sometimes associated with mild induration, as here) characteristically spares nasolabial folds.
High-resolution CT scan of advanced-stage pulmonary fibrosis demonstrating reticular opacities with honeycombing in predominantly subpleural distribution. This pattern can be present in rheumatoid arthritis–related interstitial lung disease, Sjögren syndrome, and scleroderma.
Ground-glass opacification (GGO) may correlate with active alveolitis and favorable response to therapy. GGO is among earliest features of rheumatoid arthritis–induced interstitial lung disease.
Patient with lymphocytic interstitial pneumonia.
Usual interstitial pneumonitis. Subpleural and paraseptal inflammation are present, with appearance of temporal heterogeneity. Patchy scarring of lung parenchyma and normal (or nearly normal) alveoli interspersed between fibrotic areas are hallmarks of this disease. In addition, lung architecture is completely destroyed. This pattern can be present in rheumatoid arthritis–induced interstitial lung disease and generally is associated with poor prognosis.
Table 1. Important Physical Findings in Collagen-Vascular Diseases
CVD Skin and Musculoskeletal System Lungs Heart Salivary Glands Eyes
RA* Subcutaneous nodules, digital ulcers, nail-fold infarcts Bibasilar Velcro crackles, signs of pulmonary hypertension, pleural effusion Pericarditis, myocarditis N/A N/A
SLE* Malar rash, alopecia, livedo reticularis, erythema, telangiectasia, capillary infarcts, polyarthritis Pleural effusion or rub, pneumonitis, cor pulmonale, diaphragmatic weakness Pericarditis, myocarditis, CAD N/A N/A
SD Thickening of skin of face, fingers, and hands; Raynaud phenomenon and ischemic changes of fingertips Cor pulmonale, inspiratory Velcro crackles at lung bases Restrictive pericardial disease, conduction defects, CHF N/A N/A
SS* Secondary SS can manifest similarly to RA and SLE Secondary SS can manifest similarly to RA and SLE N/A Xerostomia, parotid gland swelling Keratoconjunctivitis sicca
PM Proximal muscle weakness Respiratory muscle failure N/A N/A N/A
DM Heliotrope rash of eyelids, Gottron papules, Respiratory muscle failure N/A N/A N/A
AS Sacroiliitis Restriction in chest expansion, pulmonary apical fibrosis Aortic insufficiency N/A Anterior uveitis
* MCTD can manifest with the signs and symptoms of RA, SLE, or SS.



AS = ankylosing spondylitis; CAD = coronary artery disease; CHF = congestive heart failure; CVD = collagen-vascular disease; DM = dermatomyositis; PM = polymyositis; RA = rheumatoid arthritis; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome.



Table 2. Autoantibodies in Collagen-Vascular Diseases
Autoantibody RA SLE SD SS PM/DM AS MCTD
RF + + + + Rare - +
ANA + + + + Rare - +



(speckled)



ds-DNA - + - - - - -
Anticentromere - - + (limited) Rare Rare - -
Scl-70 - - + (diffuse)   Rare - -
Anti-Jo - - - Rare + (ILD) - -
ANCA Rare Rare - - - - -
Smith antibody - + - - - - -
Anti-Ro/SSA and anti-La/SSB - - - + - - -
Anti-U1-RNP and anti-UN-70 kd - - - - - - +
Anti-CCP + - - - - - -
ANA = antinuclear antibody; ANCA = antineutrophilic cytoplasmic antibody; AS = ankylosing spondylitis; DM = dermatomyositis; ds-DNA = double-stranded DNA antibody; ILD = interstitial lung disease; MCTD = mixed connective-tissue disease; PM = polymyositis; RA = rheumatoid arthritis; RF = rheumatoid factor; RNP = ribonucleoprotein; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; CCP = cyclic citrullinated peptide.
Table 3. Radiographic Patterns of Collagen-Vascular Diseases
Radiologic Pattern RA SLE SD Secondary SS PM/DM AS MCTD
Pleural effusion + + + ± - - +
Interstitial pneumonitis, fibrosis UIP and NSIP patterns + LIP pattern ± + Upper apical fibrosis[46] +
BOOP ± + + + + ± ±
Pulmonary nodules Rheumatoid pulmonary nodules; uncommon, may be 1-5 mm, single or multiple, may cavitate - - Follicular lymphoid hyperplasia or lymphoma can present as lung nodules + - -
Bronchiectasis + + + + + + +
Caplan syndrome Coal worker’s pneumoconiosis, rheumatoid nodules - - - - - ±
Diffuse pulmonary hemorrhage - + - - - - ±
Shrinking lung syndrome - Loss of lung volume at bases with no parenchymal pathology - - - - ±
Diaphragmatic dysfunction - + - May be present + - ±
Cysts, honeycombing 10% of patients have subpleural honeycombing; compared with IPF, it is more anterior and involves upper lobes Uncommon + Present, especially in LIP + Upper-lobe cyst may become infected with Aspergillus species -
GGO Present, especially in NSIP + + + + - ±
AS = ankylosing spondylitis; BOOP = bronchiolitis obliterans organizing pneumonia; DM = dermatomyositis; GGO = ground-glass opacification; IPF = idiopathic pulmonary fibrosis; LIP = lymphoid interstitial pneumonia; MCTD = mixed connective-tissue disease; NSIP = nonspecific interstitial pneumonia; PM = polymyositis; RA = rheumatoid arthritis; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; UIP = usual interstitial pneumonia.
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.