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Interstitial Lung Disease With
Rheumatological Diseases
MOHAMED ALFAKI
(1) pulmonary involvement may be associated with high morbidity and mortality in
this population, and
(2) pulmonary diseas...
RHEUMATOLOGIC CONDITIONS
Many different types of pulmonary manifestations may occur with each
rheumatologic condition; how...
Table 1. ILD in Rheumatological disease in children
JIA SLE JDM SSc MCTD Sarcoidosis WG MPA
Interstitial
lung disease
(ILD...
DIAGNOSTIC APPROACH IN CHILDHOOD
RHEUMATOLOGIC CONDITIONS
History
Examination
The choice of diagnostic tests depends on pr...
HRCT is a preferred imaging technique for evaluation of pediatric DLD.
Imaging
Chest radiographs (CXRs) are commonly perfo...
Pulmonary function tests
In systemic inflammatory diseases of childhood with lung involvement, PFTs
with evaluation of lun...
Table 2. Patterns of pulmonary function and gas exchange impairment
in lung disease associated with systemic inflammatory ...
Bronchoscopy with bronchoalveolar lavage
Bronchoscopy with bronchoalveolar lavage (BAL) is a commonly used
invasive techni...
BAL cytology is important for distinction of chILD from other causes of DLD,
such as aspiration (lipid-laden macrophages),...
Lung biopsy/histopathology
Genetic testing has rendered lung biopsy unnecessary in some infants with
chILD
Lung biopsy is ...
Serum and alveolar biomarkers of lung
disease in connective tissue disorders Krebs von den lungen-6 antigen (KL-6)
has bee...
Juvenile idiopathic arthritis
JIA is the most common chronic rheumatic disease in childhood
with a prevalence of 16 to 150...
Macrophage-activating syndrome, which occurs in 10% of systemic JIA
patients, is often associated with respiratory failure...
A 12-year-old child with systemic JIA diagnosed
in infancy . HRCT scan showed bilateral diffuse
interstitial changes, incl...
Prognosis is generally good in JIA with mortality well below 1%
Hashkes PJ, Wright BM, Lauer MS, et al. Mortality outcomes...
Pulmonary involvement in pediatric SLE has been reported to occur in 18% to
40% of patients within the first year of diagn...
.
Acute pneumonitis, diffuse alveolar hemorrhage, pulmonary embolism, and
pulmonary hypertension are also rare pulmonary m...
Figure 2: Bilateral diffuse bilateral ground glass
opacification, nodular markings, interlobular septal
thickening, bronch...
Shrinking lung syndrome (SLS) is also rare but may result in restrictive
lung disease mimicking ILD . In SLS, a chest CT s...
An example of shrinking lung
syndrome in a patient affected by
SLE
Treatment
Rigorous investigation and treatment of infections.
Exclude other causes.
Corticosteroids corner stone in pulmon...
Symptomatic lung involvement is rare in children with JDM which is distinct
from adult onset dermatomyositis in which symp...
ILD in adults may proceed, appear concomitantly with, or develop
after the onset of skin and muscle manifestations .In con...
The most common presenting symptoms of ILD are cough and dyspnea,
however, ILD is reported to occur without symptoms. PFTs...
A 2-year-old child who presented with fever, hypoxia,
and
persistent chest infiltrates. HRCT showed bilateral
patchy airsp...
Treatment
High dose corticosteroids
Immunosuppressive therapy methotrexate depending on
observational studies.
cyclosporin...
Systemic scleroderma
Systemic scleroderma is the prototypic CTD associated with the
development of ILD.
Pulmonary involvem...
Pulmonary involvement is often asymptomatic. Although dyspnea is the most
frequent symptom in children with lung involveme...
Antitopoisomerase I (anti-Scl-70) antibodies and anti- U3RNP
antibodies are associated with pulmonary fibrosis and poor pr...
Early ILD in teen with scleroderma. HRCT
shows typical changes of peripheral
interlobular septal thickening with fibrosis ...
Treatment
No controlled trials in juvenile SSC
Cyclophosphamide some degree of efficacy.
Lung transplant selected cases in...
MCTD
is a rare diagnosis in children that can have life-threatening pulmonary
involvement. It is characterized by the pres...
Treatment
No controlled trials available
Variable presentation so therapy should be individualized.
Better outcome in chil...
Childhood vasculitides
Vasculitis syndromes are generally classified according to their clinical
manifestations, the size ...
Classic" granulomatosis with polyangiitis is a form of systemic vasculitis
(polyangiitis) with necrotizing granulomatous i...
Pulmonary (cough, hemoptysis, dyspnea, chest pain) 70–95
Upper airway (epistaxis, sinusitis, rhinorrhea, otitis, hearing
i...
Pulmonary involvement in GPA can be asymptomatic, insidious in onset,
or severe and fulminant. Pulmonary disease may cause...
Diagnosis
Clinical and supportive serology [anti- PR3 ANCA –positive] and
histopathology.
Treatment
Standard glucocorticoi...
Microscopic polyangiitis (MPA) is a necrotizing vasculitis without
granulomatous inflammation that predominantly affects s...
CLINICAL FEATURES OF MICROSCOPIC POLYANGIITIS
Clinical Manifestations Frequency (%)
Rapidly progressive glomerulonephritis...
Sjo¨gren’s syndrome
Is so rare in children there are, however, case reports of LIP and PAH
occurring in children with Sjo¨...
Lymphocytic interstitial pneumonitis or LIP is uncommon,
being seen mainly in patients with autoimmune disease,
particular...
Sarcoidosis
Chronic inflammatory disease in which granulomatous lesion can develop in
many organs . The current concept is...
Pulmonary involvement occurs in more than 90% and pediatric cases,
commonly affecting the intrathoracic lymph nodes and th...
Progressive fibrosis in sarcoidosis
elevated serum angiotensin-converting- enzyme may provide additional
evidence of sarcoidosis.
Bronchoalveolar lavage (BAL)...
FIGURE 57-11. Bronchoscopy picture of sarcoid
airway: right upper lobe RUL airway involvement
with hypervascularity and wa...
Treatment
Difficult decision
Corticosteroids depending on adults study
Indication
Worsening pulmonary symptoms and radiolo...
Interstitial lung disease with rheumatological diseases
Interstitial lung disease with rheumatological diseases
Interstitial lung disease with rheumatological diseases
Interstitial lung disease with rheumatological diseases
Interstitial lung disease with rheumatological diseases
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Interstitial lung disease with rheumatological diseases

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highlights DLD in some Rheumatological diseases.

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Interstitial lung disease with rheumatological diseases

  1. 1. Interstitial Lung Disease With Rheumatological Diseases MOHAMED ALFAKI
  2. 2. (1) pulmonary involvement may be associated with high morbidity and mortality in this population, and (2) pulmonary disease may be the predominant initial clinical presentation in a subset of these patients.
  3. 3. RHEUMATOLOGIC CONDITIONS Many different types of pulmonary manifestations may occur with each rheumatologic condition; however, certain patterns of lung involvement are recognized with increased frequency in each condition. The most devastating pulmonary manifestation pulmonary fibrosis – may result in progressive restrictive lung disease and eventual death from respiratory failure. In the setting of known or suspected rheumatologic disorders, diagnosis and management of DLD are challenging, and require close collaboration among rheumatologists, pulmonologists, and other specialists.
  4. 4. Table 1. ILD in Rheumatological disease in children JIA SLE JDM SSc MCTD Sarcoidosis WG MPA Interstitial lung disease (ILD) + + + +++ ++ + - - Dell SD, Schneider R. Pulmonary involvement in the systemic inflammatory diseases of childhood. In Wilmott RW, Boat TF, Bush A, et al., editors. Kendig and Chernick’s disorders of the respiratory tract in children. Philadelphia: Elsevier Saunders; 2012.
  5. 5. DIAGNOSTIC APPROACH IN CHILDHOOD RHEUMATOLOGIC CONDITIONS History Examination The choice of diagnostic tests depends on presenting signs and symptoms, age of presentation, severity and progression of the disease, immunocompetence and family history, and the known or suspected underlying systemic disorder.
  6. 6. HRCT is a preferred imaging technique for evaluation of pediatric DLD. Imaging Chest radiographs (CXRs) are commonly performed in children with respiratory symptoms and suspected DLD; however, though often useful, they are neither sensitive nor specific and seldom provide specific chILD diagnoses controlled ventilation high-resolution computed tomography (CVHRCT) performed with sedation and mask ventilation or in anesthetized, intubated child is the preferred technique.
  7. 7. Pulmonary function tests In systemic inflammatory diseases of childhood with lung involvement, PFTs with evaluation of lung volumes and diffusing capacity for carbon monoxide (DLCO) are important in screening for lung disease, determining its severity, and monitoring for disease progression.
  8. 8. Table 2. Patterns of pulmonary function and gas exchange impairment in lung disease associated with systemic inflammatory conditions Pattern of ventilatory impairment Diffusing capacity for carbon monoxide Gas exchange characteristics Chest wall restriction (muscle weakness or chest wall deformity Restrictive defect with low peak flow in more severe disease Preserved until severe loss of volume With severe disease, hypoventilation results in hypercapnia and hypoxia with normal a–A gradient Pulmonary fibrosis Restrictive defect Reduced With severe disease, hypoxia at rest Bronchiectasis Obstructive defect Preserved until severe end stage disease With end-stage disease, hypoxia at rest Diffuse alveolar hemorrhage Variable – often restrictive Increased if hemorrhage is recent During active bleeding, hypoxia, often profound with a wide a–A gradient Pulmonary vascular disease Normal pulmonary function tests Reduced Hypoxia at rest even with moderate pulmonary hypertension Mixed disease: pulmonary fibrosis and muscle weakness Restrictive defect, often severe Less reduced than expected for degree of restrictive defect Hypoxia at rest or with exercise is frequent Dell SD, Schneider R. Pulmonary involvement in the systemic inflammatory diseases of childhood. In Wilmott RW, Boat TF, Bush A, et al., editors. Kendig and Chernick’s disorders of the respiratory tract in children. Philadelphia: Elsevier Saunders; 2012.
  9. 9. Bronchoscopy with bronchoalveolar lavage Bronchoscopy with bronchoalveolar lavage (BAL) is a commonly used invasive technique in patients with ILD, it is helpful for distinguishing chILD and autoimmune lung disease from infection. Airway abnormalities suggestive of necrotizing granulomatosis vasculitis (NGV) or sarcoidosis may be identified.
  10. 10. BAL cytology is important for distinction of chILD from other causes of DLD, such as aspiration (lipid-laden macrophages), pulmonary hemorrhage syndromes (hemosiderin-laden macrophages), pulmonary alveolar proteinosis (PAP) [periodic acid-Schiff (PAS)-positive material in alveolar macrophages], pulmonary histiocytosis (CD-1a cells), lysosomal storage disorders, and sarcoidosis. Cytology count differentials may help to narrow the differential diagnosis of lung disease. Vece TJ, Fan LL. Diagnosis and management of diffuse lung disease in children [review]. Paediatr Respir Rev 2011; 12:238–242.
  11. 11. Lung biopsy/histopathology Genetic testing has rendered lung biopsy unnecessary in some infants with chILD Lung biopsy is still indicated in infants with rapidly progressive or severe disease when establishing a diagnosis is needed to guide the treatment options including lung transplant. In Rheumatologic disorders lung biopsy is reserved for a subset of patients when a diagnostic dilemma remains despite other diagnostic testing, particularly if histopathology may change the management. Nogee LM. Genetic basis of children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol 2010; 23:15–24.
  12. 12. Serum and alveolar biomarkers of lung disease in connective tissue disorders Krebs von den lungen-6 antigen (KL-6) has been identified as a serum marker of ILD. KL-6 occurs on the surface of alveolar cells; however, its serum levels increase when type II pneumocytes proliferate or capillary integrity is disturbed. Increased serum levels of KL-6 have been found in patients with active interstitial pneumonitis associated with CTDs. Doishita S, Inokuma S, Asashima H, et al. Serum KL-6 level as an indicator of active or inactive interstitial pneumonitis associated with connective tissue diseases. Intern Med 2011; 50:2889–2892.
  13. 13. Juvenile idiopathic arthritis JIA is the most common chronic rheumatic disease in childhood with a prevalence of 16 to 150 per 100,000. Lung involvement, with the exception of transient pleuritis which associated most times with pericarditis is sufficiently rare in JIA to warrant investigation. Other rare associations with JIA include bronchiolitis obliterans organizing pneumonia (BOOP), pulmonary hemosiderosis and lymphocytic interstitial pneumonitis (LIP). Sohn DI, Laborde HA, Bellotti M, Seijo L. Juvenile rheumatoid arthritis and bronchiolitis obliterans organized pneumonia. Clin Rheumatol 2007; 6:247–250.
  14. 14. Macrophage-activating syndrome, which occurs in 10% of systemic JIA patients, is often associated with respiratory failure requiring ventilator support. The most severe pulmonary manifestations have been seen with the systemic and polyarticular subtypes. lipoid pneumonia in children with systemic JIA, not secondary to aspiration, is a rare complication associated with severe, refractory disease. Schultz R, Mattila J, Gappa M, Verronen P. Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemic juvenile arthritis (CJA). Pediatr Pulmonol 2001; 32:397–402.
  15. 15. A 12-year-old child with systemic JIA diagnosed in infancy . HRCT scan showed bilateral diffuse interstitial changes, including thickening of the interlobular septa and areas of fibrosis and ground-glass densities.
  16. 16. Prognosis is generally good in JIA with mortality well below 1% Hashkes PJ, Wright BM, Lauer MS, et al. Mortality outcomes in pediatric rheumatology in the US. Arthritis Rheum 2010; 62:599–608. Treatment NSAID Methotrexate TNF Inhibitors –infliximab IL-1 AND 6 inhibitors. Corticosteroids and cyclosporine for refractory diseases with pulmo. HTN .
  17. 17. Pulmonary involvement in pediatric SLE has been reported to occur in 18% to 40% of patients within the first year of diagnosis and in 18% to 81% of patients at any time during the disease course. Systemic lupus erythematosus Pleuritis with or without pleural effusion occurs in 9–32% of children at initial disease presentation and is generally quickly reversible with steroid therapy. Silverman E, Eddy A. Systemic lupus erythematosus. In Cassidy JT, Petty RE, Laxer R, Lindsley C, editors. Textbook of pediatric rheumatology. 6th ed. Philadelphia: Elsevier; 2011. pp. 318. .Rood MJ, ten Cate R, van Suijlekom-Smit LW, et al. Childhood-onset systemic lupus erythematosus: clinical presentation and prognosis in 31 patients. Scand J Rheumatol 1999; 28:222–226
  18. 18. . Acute pneumonitis, diffuse alveolar hemorrhage, pulmonary embolism, and pulmonary hypertension are also rare pulmonary manifestations in SLE. Swigris JJ, Fischer A, Gilles J, et al. Pulmonary and thrombotic manifestations of systemic lupus erythematosus. Chest 2008; 133:271–280. Chronic ILD due to SLE is extremely rare but can occur .in a necropsy series of 90 lupus patients, none had acute or chronic pneumonitis. If chronic pneumonitis does occur, Sjogren's syndrome, infection, or drug toxicity should be excluded. Quadrelli SA, Alvarez C, Arce SC, et al. Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies. Lupus 2009; 18:1053–1060. Beresford MW, Cleary AG, Sills JA, et al. Cardio-pulmonary involvement in juvenile systemic lupus erythematosus. Lupus 2005; 14:152–158.
  19. 19. Figure 2: Bilateral diffuse bilateral ground glass opacification, nodular markings, interlobular septal thickening, bronchiectatic changes and honeycombing pattern suggestive of interstitial lung disease.
  20. 20. Shrinking lung syndrome (SLS) is also rare but may result in restrictive lung disease mimicking ILD . In SLS, a chest CT shows normal lung parenchyma, whereas ultrasound or electrophysiological studies may show diaphragmatic dysfunction. SLS usually responds to corticosteroid therapy and is associated with a good prognosis Opportunistic infections are a leading cause of death in children with lupus, so exclusion of infection is necessary before attributing respiratory symptoms to inflammatory disease activity Ferguson PJ, Weinberger M. Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythematosus. Pediatr Pulmonol 2006; 41:194–197. Oud KTM, Bresser P, Berge RJMt, Jonkers RE. The shrinking lung syndrome in systemic lupus erythematosus: improvement with corticosteroid therapy. Lupus 2005; 14:959–963. Wang LC, Yang YH, Lu MY, Chiang BL. Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect 2003; 36:203–208.
  21. 21. An example of shrinking lung syndrome in a patient affected by SLE
  22. 22. Treatment Rigorous investigation and treatment of infections. Exclude other causes. Corticosteroids corner stone in pulmonary manifestation. Immunosuppressive agents used as corticosteroids sparing therapy. Plasmapheresis .
  23. 23. Symptomatic lung involvement is rare in children with JDM which is distinct from adult onset dermatomyositis in which symptomatic lung involvement occurs in more than half of the patients . Fathi M, Lundberg IE, Tornling G. Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med 2007; 28:451–458. Juvenile dermatomyositis Rare autoimmune inflammatory myositis. There are, however, case reports of acute-onset, steroid-refractory, and rapidly progressive fatal ILD in children, usually also associated with an air leak syndrome Prahalad S, Bohnsack JF, Maloney CG, Leslie KO. Fatal acute fibrinous and organizing pneumonia in a child with juvenile dermatomyositis. J Pediatrics 2005; 146:289–292. 56. Yamanishi Y, Maeda H, Konishi F, et al. Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. Scand J Rheumatol 1999; 28:58–61.
  24. 24. ILD in adults may proceed, appear concomitantly with, or develop after the onset of skin and muscle manifestations .In contrast, symptomatic pulmonary involvement is infrequent in children. In studies of adult patients with myositis, the presence of anti-Jo-1 antibodies and antisynthetase antibodies is associated with ILD and also may be associated with severe disease in children. Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991; 70:360– 374.
  25. 25. The most common presenting symptoms of ILD are cough and dyspnea, however, ILD is reported to occur without symptoms. PFTs show a restrictive ventilatory defect, with decreased lung volumes, reduced diffusing capacity for carbon monoxide, and a normal or elevated FEV1:FVC ratio. Pneumomediastinum ,aspiration pneumonia and hypoventilation are recognized complication in adults.
  26. 26. A 2-year-old child who presented with fever, hypoxia, and persistent chest infiltrates. HRCT showed bilateral patchy airspace consolidation with air bronchograms. A lung biopsy confirmed the diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP). Six months later the child developed myalgias and heliotrope rash and was diagnosed with JDM.
  27. 27. Treatment High dose corticosteroids Immunosuppressive therapy methotrexate depending on observational studies. cyclosporine Severe or life threatening disease like ILD IV cyclophosphamide plus steroids. intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety.Riley P, Maillard SM, Wedderburn LR, Woo P, Murray KJ, Pilkington CA Rheumatology (Oxford). 2004 Apr; 43(4):491-6.
  28. 28. Systemic scleroderma Systemic scleroderma is the prototypic CTD associated with the development of ILD. Pulmonary involvement is the leading cause of mortality in SSc. Ferri C, Valentini G, Cozzi F, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 2002; 81:139–153. Manifestations are ILD and pulmonary arterial hypertension (PAH). ILD occurs in about 50% of children with SSc, whereas PAH is relatively rare (5–8%) in children compared with adults
  29. 29. Pulmonary involvement is often asymptomatic. Although dyspnea is the most frequent symptom in children with lung involvement, it only occurs in 10% to 26% of children with SSc at presentation or during the disease course. Chest imaging classically shows bibasilar fibrosis with or without traction bronchiectasis and honeycombing Schurawitzki H, Stiglbauer R, Graninger W, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176:755–759.
  30. 30. Antitopoisomerase I (anti-Scl-70) antibodies and anti- U3RNP antibodies are associated with pulmonary fibrosis and poor prognosis. Arthritis Res Ther. 2003; 5(2): 80–93. Published online 2003 Feb 12.
  31. 31. Early ILD in teen with scleroderma. HRCT shows typical changes of peripheral interlobular septal thickening with fibrosis and traction bronchiectasis.
  32. 32. Treatment No controlled trials in juvenile SSC Cyclophosphamide some degree of efficacy. Lung transplant selected cases in adult. Autologous stem cell transplant under evaluation.
  33. 33. MCTD is a rare diagnosis in children that can have life-threatening pulmonary involvement. It is characterized by the presence of high titer anti-U1 ribonucleoprotein RNP) antibodies in combination with clinical features of SLE, SSc, and/or dermatomyositis and was first described as a distinct clinical phenotype in 1972. Pulmonary disease is a major source of morbidity and mortality in adults with MCTD, occurring in about 75% of adult patients.Case series of MCTD in children suggest a similar frequency of pulmonary involvement , although pulmonary hypertension seems to be less common and lung disease is generally mild. Mier RJ, Shishov M, Higgins GC, et al. Pediatric-onset mixed connective tissue disease. Rheum Dis Clin N Am 2005; 31:483–496; vii.
  34. 34. Treatment No controlled trials available Variable presentation so therapy should be individualized. Better outcome in children.
  35. 35. Childhood vasculitides Vasculitis syndromes are generally classified according to their clinical manifestations, the size and type of blood vessels involved, and the pathologic features found within the vessel walls. Pulmonary involvement occurs with most of vasculitis syndromes, with clinically significant involvement with positive ANC antibodies. Small vessels necrosis with pulmonary and renal involvement.
  36. 36. Classic" granulomatosis with polyangiitis is a form of systemic vasculitis (polyangiitis) with necrotizing granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis . GPA [ Wegener’s granulomatosis]
  37. 37. Pulmonary (cough, hemoptysis, dyspnea, chest pain) 70–95 Upper airway (epistaxis, sinusitis, rhinorrhea, otitis, hearing impairment, ear pain, destructive lesions/bony deformities ulcerations) 70–95 Tracheobronchial (subglottic stenosis, bronchial stenosis, endobronchial lesion) 10–55 Renal/glomerulonephritis 50–85 Cutaneous (purpura, ulcers, vesicles, or nodules) 45–60 Musculoskeletal (arthralgias, myalgias, arthritis) 30–70 Ocular (conjunctivitis, uveitis, episcleritis, scleritis, proptosis) 25–55 Constitutional (fever, weight loss, arthralgias, malaise) 15–45 Nervous system (peripheral, central, headache) 10–30 Cardiac (coronary vasculitis, pericarditis) 5–15 CLINICAL FEATURES OF WEGENER'S GRANULOMATOSIS : Am Thorac Soc. 2006 Mar; 3(1): 48 57. doi: 10.1513/pats.200511-120JH
  38. 38. Pulmonary involvement in GPA can be asymptomatic, insidious in onset, or severe and fulminant. Pulmonary disease may cause any of the following: Pulmonary infiltrates (71%) Cough (34%) Hemoptysis (18%) Chest discomfort (8%) Dyspnea (7%) [1] Diffuse alveolar hemorrhage due to alveolar capillaritis (5%-45%) Atelectasis, with dullness on percussion, decreased breath sounds, and crackles on auscultation Manganelli P, Fietta P, Carotti M, Pesci A, Salaffi F. Respiratory system involvement in systemic vasculitis. Clin Exp Rheumatol. March-April 2006. 24:S48-S59. Fauci AS, Haynes BS, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. January 1983. 98(1):76-85.
  39. 39. Diagnosis Clinical and supportive serology [anti- PR3 ANCA –positive] and histopathology. Treatment Standard glucocorticoids and cyclophosphamide CPA. Methotrexate Rituximab for refractory disease. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. March 2009. 68:310-317.
  40. 40. Microscopic polyangiitis (MPA) is a necrotizing vasculitis without granulomatous inflammation that predominantly affects small vessels (ie, capillaries, venules, or arterioles) and can present with pulmonary capillaritis or in the context of interstitial lung disease .
  41. 41. CLINICAL FEATURES OF MICROSCOPIC POLYANGIITIS Clinical Manifestations Frequency (%) Rapidly progressive glomerulonephritis 100 Pulmonary (hemorrhage, hemoptysis) 10–30 Constitutional symptoms (fever, chills, weight loss, arthralgias/myalgias) 70–80 Cutaneous (purpura, urticaria, subcutaneous nodules, exanthem) 50–65 Nervous system (mononeuritis muliplex) 15–50 GI (pain, GI bleeding, infarction, perforation) 30–45 Ocular (conjunctivitis, uveitis) 0–30 Cardiac 10–20 Upper airway 0–15 Am Thorac Soc. 2006 Mar; 3(1): 48 57. doi: 10.1513/pats.200511-120JH
  42. 42. Sjo¨gren’s syndrome Is so rare in children there are, however, case reports of LIP and PAH occurring in children with Sjo¨gren’s syndrome. Houghton KM, Cabral DA, Petty RE, Tucker LB. Primary Sjogren’s syndrome in dizygotic adolescent twins: one case with lymphocytic interstitial pneumonia. J Rheumatol 2005; 32:1603–1606. Theander E, Henriksson G, Ljungberg O, et al. Lymphoma and other malignancies in primary Sjogren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 2006; 65:796– 803. Pulmonary involvement occurs in up to 75% of adult patients with Sjogren's. Small airways obstructive disease is caused by lymphocytic inflammation around the bronchioles and bronchi. Most lung diseases are mild and no progressive. LIP and lymphoma are less common but serious pulmonary manifestations
  43. 43. Lymphocytic interstitial pneumonitis or LIP is uncommon, being seen mainly in patients with autoimmune disease, particularly Sjogren's syndrome
  44. 44. Sarcoidosis Chronic inflammatory disease in which granulomatous lesion can develop in many organs . The current concept is that a still unknown stimulus activates quiescent T cells and macrophages leading to recruitment and activation of mononuclear cells. Granuloma formation. Pulmonary fibrosis and blindness are two potential long-term morbidities that call for careful consideration for treatment and follow-up of the sarcoidosis patient.
  45. 45. Pulmonary involvement occurs in more than 90% and pediatric cases, commonly affecting the intrathoracic lymph nodes and the pulmonary parenchyma. Symptoms include cough, dyspnea and wheeze. Exam may be normal or showing crackles. Radiology 0 I II III VI
  46. 46. Progressive fibrosis in sarcoidosis
  47. 47. elevated serum angiotensin-converting- enzyme may provide additional evidence of sarcoidosis. Bronchoalveolar lavage (BAL) cell profiles are not specific for sarcoidosis, but they may help to narrow the differential diagnosis. BAL shows a lymphocytosis in > 85%; neutrophils are normal or low except in late disease; CD4:CD8 ratio is increased (opposite to findings in ILD associated with connective tissue diseases) in 50% to 60%. BAL cell profile is not helpful in monitoring disease progression or response to therapy.
  48. 48. FIGURE 57-11. Bronchoscopy picture of sarcoid airway: right upper lobe RUL airway involvement with hypervascularity and waxy nodules
  49. 49. Treatment Difficult decision Corticosteroids depending on adults study Indication Worsening pulmonary symptoms and radiological changes. Cardiac, neurological, ocular and renal symptoms. Sever debilitating symptoms.

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