Collagen-Vascular Disease Associated With Interstitial Lung Disease 

  • Author: Arshad Ali, MD; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: May 31, 2011
 

Background

Collagen-vascular diseases (CVDs) are a heterogeneous group of autoimmune disorders of unknown etiology. Many CVDs involve the lungs directly or involve them as a complication of treatment of the CVD. Several different components of the respiratory system may be involved, including airways, vessels, parenchyma, pleura, and/or respiratory muscles. Interstitial lung disease (ILD) is the most common pulmonary complication of the CVDs. CVDs include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS) or scleroderma (SD), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjögren syndrome (SS), and mixed connective-tissue disease (MCTD).[1]

Felizardo et al[2] conducted a study to find out the incidence of ILD in CVD patients in an outpatient setting. They found that 30% patients had ILD, and in these patients, the distribution of CVDs was that 8% had SD, 1% had CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome, 34% had RA, 4.3% had SLE, 6.4% had DM, 10.6% had primary syndromes, and 8.5% had secondary SS.

The incidence of ILD is increasing in CVD patients.[3] This can be mainly attributed to the increased use of invasive techniques such as bronchoscopy and video-assisted thoracoscopic surgery and, in part, to the use of high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs). Because the prognosis, degree of reversibility, and optimal therapy differ for each disease presentation, a thorough knowledge of the pulmonary clinical picture of each connective-tissue disease is important.

Related eMedicine articles include Systemic Lupus Erythematosus,Rheumatoid Arthritis,Systemic Sclerosis,Scleroderma,Dermatomyositis/Polymyositis,Ankylosing Spondylitis,Sjogren Syndrome, and Mixed Connective-Tissue Disease.

Next

Pathophysiology

The etiology and pathogenesis of connective-tissue diseases is unknown. The role of autoimmunity in ILDs associated with connective-tissue disorders such as systemic sclerosis, SLE, and RA is well established.[4]

CVD can cause a variety of pathological lung abnormalities, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), organizing pneumonia, diffuse alveolar damage, lymphoid interstitial pneumonia (LIP), bronchiectasis, constrictive bronchiolitis, follicular bronchiolitis, alveolar hemorrhage, pulmonary hypertension,[5] and drug-induced lung disease. The frequency of each lung disease is different among underlying CVDs. Some of the pathologic characteristics of CVDs are as follows:

  • Lungs are involved in more than 50% of patients with systemic sclerosis. A diffuse interstitial and alveolar fibrosis may appear with variable fibrous thickening of small pulmonary vessels.
  • Pleuritis and pleural effusions are the most common pulmonary manifestations of SLE. Less commonly, evidence of alveolar injury in the form of edema and hemorrhage is present. In some cases, chronic interstitial fibrosis is noted.
  • The most common interstitial pathologic pattern in patients with RA is diffuse fibrosis with inflammation.[6] Follicular bronchiolitis (ie, development of the lymphoid follicles with germinal centers) can be associated with significant airway obstruction. Pulmonary vasculitis[7] causing pulmonary hypertension is also reported.
  • The pulmonary complications of PM/DM may occur by 3 mechanisms: primary interstitial pneumonitis, aspiration pneumonia secondary to hypotonic esophagus, and hypostatic pneumonia secondary to chest wall involvement.

Risk factors for the development of CVDs are as follows:

  • Genetic susceptibility can play a role. Many individuals are predisposed genetically to develop CVDs. For example, in persons with SD class II, major histocompatibility complex associations increase the risk of interstitial pulmonary fibrosis (IPF). A genetic predisposition is found in persons with SS, including familial clustering and an association with HLA-Dw2 and HLA-Dw3. Further, AS is strongly associated with HLA-B27.[8]
  • Hormonal influence can be a factor. Pregnancy exacerbates various CVDs, including SLE and RA, suggesting that hormones (especially estrogen) play an important role.[9]
  • High titers of rheumatoid factor (RF) and the presence of rheumatoid nodules are also associated with an increased prevalence of pulmonary fibrosis in persons with RA.
  • Cigarette smoking (>25 pack-year) increases the risk of ILD in patients with RA.[10]
Previous
Next

Epidemiology

Frequency

United States

The exact frequency of CVD is unknown. The incidence and prevalence vary depending on various epidemiological studies. However, the fact that 10-90% of patients with CVDs (depending on the type of CVD) will have lung involvement in their lifetime is well known.

  • In the United States, SLE is a fairly common autoimmune disease and affects 1-2 in 2000 people.[11]
  • RA affects 0.3-1.5% of the North American population.
  • The prevalence of SD is 26 cases per 100,000 population in the United States.[12, 13, 14]
  • The prevalence of SS is 0.5-0.3%.
  • PM/DM is relatively rare, affecting 2-3 in 100,000 people.[15]

International

  • RA is a common disease with a worldwide prevalence of approximately 1% and an annual incidence of approximately 3 in 10,000 adults.[16, 17]
  • SS affects 1 in 1250 individuals.[18]
  • SLE affects approximately 1 in 2000 individuals.[19]
  • PSS and limited SD affect 1 in 5,000-20,000 individuals.
  • PM and DM are relatively rare; they affect 1 in 100,000-200,000 individuals per year in the world.

Mortality/Morbidity

In general, CVD-associated ILD causes significant morbidity and mortality. PM/DM and systemic sclerosis have high mortality rates compared with other CVDs. In one study, the diagnostic Kaplan-Meier analysis showed that patients with PM/DM and those with systemic sclerosis did not survive as long as patients with other types of CVD.

Mortality is high in patients with CVD who develop ILD and pulmonary hypertension. Takizawa et al[20] found that in 715 patients with CVDs, ILD and pulmonary hypertension were found to be important causes of death (37.5% and 6%, respectively).

In patients with RA and SLE who develop ILD, the mortality rate is 3-4 times higher compared with the general population.

Race

RA is more common in adult whites. The prevalence of SLE is higher among Asians, African Americans, and Hispanic Americans.[21]

Sex

SLE, RA, PM/DM, SS, and MCTD are more common in females. However, note that RA occurs predominantly in women but RA-related ILD is more common in middle-aged men. AS is more common in men, with a male-to-female ratio of 3:1.

Age

In general, CVDs are rare in children; the primary peak age of onset is middle age (ie, for RA, SLE, PSS, and AS). DM affecting older individuals (ie, >50-60 y) may be associated with malignancy.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

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, and American Medical Association

Disclosure: Nothing to disclose.

Hina Arif, MD  Research Assistant, Interfaith Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

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

Disclosure: Nothing to disclose.

References

  1. Fiedorczyk M, Rojewska J, Kowal-Bielecka O, Sierakowski S. Interstitial lung disease related to systemic connective tissue diseases. Przegl Lek. 2005;62(12):1471-4. [Medline].

  2. Felizardo M, Aguiar M, Mendes A, Moniz D, Sotto-Mayor R, Almeida A. Collagen vascular diseases and lung: Characterization of the outpatients with intersticial lung disease. Rev Port Pneumol. Nov;2005;(6 Suppl 1):26-7. [Medline].

  3. 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].

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

  5. Manganelli P, Salaffi F, Carotti M, Delsante G, Mozzani F. Pulmonary hypertension in rheumatic diseases. Minerva Med. Mar;1999;90(3):59-72. [Medline].

  6. 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].

  7. Feigin DS. Vasculitis in the lung. J Thorac Imaging. Jan; 1988;3(1):33-48. [Medline].

  8. 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].

  9. 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].

  10. 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].

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

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

  13. 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].

  14. 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].

  15. 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].

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

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

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

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

  20. 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].

  21. 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].

  22. 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].

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

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

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

  26. 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].

  27. 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].

  28. 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].

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

  30. 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].

  31. 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].

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

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

  34. 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].

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

  36. 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].

  37. 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].

  38. 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].

  39. 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].

  40. 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].

  41. 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].

  42. 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].

  43. 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].

  44. 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].

  45. 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].

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

  47. 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].

  48. 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].

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

  50. 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].

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

  52. 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].

  53. 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].

  54. 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].

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

  56. 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].

 
Previous
Next
 
Pulmonary hypertension is a complication of various collagen-vascular diseases. The image shows a lung biopsy specimen demonstrating severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of a pulmonary arteriole compatible with pulmonary hypertension.
Heliotrope rash in a woman with dermatomyositis.
Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.
The classic malar rash, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds.
High-resolution CT scan of advanced-stage of pulmonary fibrosis demonstrating reticular opacities with honeycombing, with predominant subpleural distribution. This pattern can be present in rheumatoid arthritis-related interstitial lung disease, Sjogren syndrome, and scleroderma.
Ground-glass opacification (GGO) may correlate with active alveolitis and a favorable response to therapy. GGO is one of the earliest features of rheumatoid arthritis-induced interstitial lung disease.
Chest radiograph of a patient with lymphocytic interstitial pneumonia.
Usual interstitial pneumonitis. Subpleural and paraseptal inflammation are present, with an appearance of temporal heterogeneity. Patchy scarring of the lung parenchyma and normal, or nearly normal, alveoli interspersed between fibrotic areas are the hallmarks of this disease. In addition, the 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 Findings in the Physical Examination of CVD Patients
CVDsSkin and Musculoskeletal SystemLungsHeartSalivary GlandsEyes
RASubcutaneous nodules, digital ulcers, nail fold infarctsBibasilar Velcro crackles, signs of pulmonary hypertension, pleural effusionPericarditis and myocarditisN/AN/A
SLEMalar rash, alopecia, livedo reticularis, erythema, telangiectasia, capillary infarcts, polyarthritisPleural effusion or rub, pneumonitis, cor pulmonale, diaphragmatic weaknessPericarditis, myocarditis, and coronary artery diseaseN/AN/A
SDThickening of the skin of face, fingers, and hands; Raynaud phenomenon and ischemic changes of the finger tipsCor pulmonale, inspiratory Velcro crackles at the lung basesRestrictive



pericardial disease, conduction defects, congestive heart failure



N/AN/A
SSSecondary SS can manifest similar to RA and SLESecondary SS can manifest similar to RA, SLEN/AXerostomia, parotid gland swellingKeratoconjunctivitis sicca
PolymyositisProximal muscle weaknessRespiratory muscle failureN/AN/AN/A
DMHeliotrope rash of eyelids, Gottron papules,Respiratory muscle failureN/AN/AN/A
SpondylitisSacroiliitisRestriction in chest expansion, pulmonary apical fibrosisAortic insufficiencyN/AAnterior



uveitis



*MCTD can manifest with the signs and symptoms of RA, SLE, or SS.
Table 2. Autoantibodies in CVDs
AutoantibodyRASLESDSSPM/DMASMCTD
RF++++Rare-+
ANA*++++Rare-+



(speckled)



ds-DNA†-+-----
Anticentromere--+ (limited)RareRare--
Scl- 70--+ (diffuse)Rare--
Anti- Jo---Rare+ (ILD)--
ANCA‡RareRare-----
Smith antibody-+-----
Anti-Ro/SSA and anti-La/SSB---+---
Anti-U1-RNP§ and Anti-UN-70 kd------+
*Antinuclear antibody



†Double-stranded DNA antibody



‡Antineutrophilic cytoplasmic antibody



§Antiribonucleoprotein antibody



Table 3. Summary of the Radiographic Patterns of CVDs
Radiological PatternRASLESDSecondary SSPM/DMASMCTD
Pleural Effusion+++±--+
Interstitial pneumonitis and fibrosisUIP and NSIP patterns+LIP



pattern



±+Upper apical fibrosis[38] +
BOOP±++++±±
Pulmonary NodulesRheumatoid 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 SyndromeCoal worker’s pneumoconiosis and 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; more anterior and involves upper lobes compared with IPF.Uncommon+Present, especially in LIP+Upper lobe cyst



may become infected with Aspergillus species



-
GGOPresent, especially in NSIP++++-±
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.