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INFLAMMATORY ARTHRITIS HRCT CHEST
- 1. INFLAMMATORY ARTHRITIS HRCT CHEST DR. PANKAJ SAINI RADIOLOGY & MEDICAL IMAGING
- 2. PURVIEW • Chest findings - challenge in both diagnosis and management • Contribute to significant morbidity and mortality • Manifestation or a presenting feature of the collagen vascular disease • Practical approach • History, physical examination, radiological, laboratory & histopathological • Screening and Surveillance • Demographic, Serological, Biomarkers • Established and emerging treatment
- 3. INTERSTITIAL LUNG DISEASE – CONNECTIVE TISSUE DISEASE • NSIP – Non specific Interstitial pneumonia – chronic progression • UIP - Usual interstitial pneumonia – chronic progression • LIP - Lymphocytic interstitial pneumonia – chronic progression • OP – Organizing pneumonia (BOOP) – subacute progression • AIP – Acute interstitial pneumonia (DAD) – acute progression • DIP – Desquamative interstitial pneumonia • FA – Fibrosing alveolitis with apical predominance Tracy J. Doyle, CHEST 2017; 152(6):1283-1295
- 4. COLLAGEN VS. DISEASE – COMMONLY AFFECTED IN ILD • Rheumatoid arthritis – RA – Associated with NSIP • Progressive systemic sclerosis – SSc / Scleroderma • Dermatomyositis / Auto immune myositis- AIM – Associated with DAD • Systemic lupus erythematosus – SLE – Associated with Diffuse alveolar damage • Sjogrens syndrome – associated with LIP • Undifferentiated rheumatoid disorders • Rheumatic related UIP similar mortality as IPF (Idiopathic pulmonary fibrosis) • Histological pattern may overlap AIM – overlap of NSIP and OP
- 5. HRCT • Narrow slice thickness (1mm) at 10mm interval • High spatial resolution image reconstruction • End inspiratory view • Window width of 1300 with Window Level of - 600 HU • Mediastinal window width 45, level of 420 width.
- 6. HRCT CHEST – “INFLAMMATORY ARTHRITIS” • Advancements in imaging technology have improved our ability to diagnose what once was thought to be a relatively rare complication is now suspected to affect 20–30% of RA patients • Commonest with SSc, RA, SLE and least common in Sjogren’s • Males predominate with RA 43.7% while female predominance in SLE 41.4% • Interstitial pattern (40%), Pleural fibrosis (28%) Consolidation (18.7%) • DAD superimposed on underlying pulmonary fibrosis • Smokers – Desquamative / respiratory bronchiolitis
- 7. HRCT FINDINGS IN ILD • Reticular – Thickened inter and intra lobular septae • Infiltration – fibrosis, abnormal cells or fluid • Interlobular septal thickening – smooth or irregular • Fine reticular pattern • Honey combing • late stage cystic air space surrounded by irregular walls RadioGraphics 2012;32:33–50 •Published online10.1148/rg.321105058
- 8. RETICULAR – THICKENED INTER AND INTRALOBULAR SEPTA • Fine, Coarse thickening - Width • Fluid, Fibrous, Infiltrates • Many conditions • Pulmonary edema • Lymphanngitis carcin’ • Alveolar proteinosos • IPF • Drug related disease • Amylodois • Collagen vascular dis’
- 9. INFILTRATION – FIBROSIS, ABNORMAL CELLS OR FLUID
- 10. INFILTRATION – FIBROSIS, ABNORMAL CELLS OR FLUID • Image
- 11. INTERLOBULAR SEPTAL THICKENING – SMOOTH OR IRREGULAR • Smooth • Pulmonary edema • Pulmonayr hemorrhage • Lymphangitis carcinomatosis • Lymphoma • Nodular • Sarcoidosos • Amylodosis • Lymphangitis carcinomatosis • Irregular • Fibrosis
- 12. • Fine thickening of the inter and intra-lobular septae • With or without GGO • Pulmonary edema • Sarcoidosis FINE RETICULAR PATTERN
- 13. HONEY COMBING – LATE STAGE CYSTIC AIR SPACE SURROUNDED BY IRREGULAR WALLS • Image
- 14. NON SPECIFIC INTERSTITIAL PNEUMONIA – NSIP • Chronic progressive ILD commonly seen especially in AIM & SSc • Better prognosis • Fibrotic NSIP May prognosctically parallel UIP • Radiological diagnosis may need to be histo-pathologically confirmed • Cellular NSIP – Ground Glass Density No honeycombing / tractional bronchiectasis / Infection • Empirical anti-inflammatory therapy
- 15. CRAZY PAVING PATTERN • Acute exacerbation of chronic progressive ILD • GGO with / out inter and intralobular septal thickening • Diffuse alveolar damage • Fibrotic changes – bronchiectasis, reticulation, honeycombing • D/D – opportunistic infection – drug induced disease • Smoking – Desquamative / bronchiolitis
- 16. USUAL INTERSTITIAL PNEUMONIA - UIP • Commonly seen especially in RA • Poor prognosis – 5 year mortality of >50% • Present in upto 60% cases of RA associated ILD – RA-ILD • Disease modifying anti rheumatic drugs - DMARD • Mycophenolate mofetil – MMF • Azathioprine – AZA • PANTHER-IPF Trial – evaluates anti- inflammatory therapy with deleterious results in UIP related to IPF raises similar concerns with UIP related to RA • Prospective trials with anti-fibrotic agents • Referral for transplant
- 17. ORGANIZING PNEUMONIA - BOOP • Commonly seen especially in AIM particularly in anti-synthetase syndrome • Intraluminal fibrosis in distal airway with associated interstitial inflammation • Radiologically – consolidation, nodules • Unique features – atoll sign / reverse halo sign • Central GGO with surrounding denser air space consolidation in an crescentic / ring shape • Worse prognosis in OP related to RA as compared to cryptogenic OP
- 18. ORGANIZING PNEUMONIA - OP OP in a previously well individual with or without new systemic complaints will merit a detailed investigation for underlying autoimmune disorder – especially anti- synthetase syndrome
- 19. ANTI-SYNTHETASE SYNDROME • Historical / Physical • very high rates of ILD • Grotton’s nodules – extensor surface • Common Imaging findings • Pneumo mediastinum has been noted in this population, as well as in patients with dermatomyositis without MDA5 (melanoma diff associated pr 5) • Limited responsie & necessitates early aggressive therapy - Transplanation • Typical serological attributes • presence of antibodies to
- 20. LYMPHOCYTIC INTERSTITIAL PNEUMONIA - LIP • Commonly seen in Sjogrens syndrome • may complicate RA & SSc • Lympho-plamacytic infiltrates • nodules and lymphoid follicles • Nodules may appear inflammatory in LIP but more likely Lymphoma • MALT – mucosa associated lymphoid tissue • RA and Sjogrens syndrome are prone to develop Lymphoma • Biopsy is warranted
- 21. PULMONARY MANIFESTATIONS IN RHEUMATOID A. • Follicular bronchiolitis • Constrictive bronchiolitis • Obstructive bronchiolitis • Bronchiectasis • Emphysema • Coexist with ILD especially in RA • Airway changes in RA especially upper airway in exposure to antigens (citrulline) & autoimmune response may initiate or propagate RA • ACPA – Anti citrulillinated protein antibody
- 22. PULMONARY MANIFESTATIONS IN RA • Bronchiectasis is common with RA • Increase risk of secondary infection especially when RA is treated with biological agents • Follicular bronchiolitis – Obstruction on PFT with declining respiratory status • Air trapping / Mosaicism on HRCT raise possibility of reversible and treatable disease and less likely obliterative bronchiolitis (not responsive unless the offending drug is identified and removed) • Concomitant emphysema – previous smoking history
- 23. PULMONARY NODULES • D/D – opportunistic infection • Nocardia, Cryptocous, Aperrgillus, AMTB • Neoplasm – Carcinoma / Lymphoma • Metabolic – Amyloidosis • Immunological – Rheumatoid Nodule • Cavitate – 50% • Often asymptomatic may often rupture • Pneumothorax, hemoptysis & infection • Inflammatory nodules in RA & Sjogrens syndrome May mimic malignancy – Mandate biopsy • “Caplans Syndrome” with Pneumoconiosis
- 24. PLEURAL INFLAMMATION AND EFFUSION • Commonly seen in RA, SLE and occasionally Systemis sclerosis & Sjogrens syndrome • Exudative effusions with low glucose and pH value in RA • “Shrinking lung” syndrome in SLE is likely due to persistent pleural inflammation
- 25. SSC – SYSTEMITC SCLEROSIS / SCLERODERMA • Common Imaging findings • > 50 % patients with earlier development • NSIP / UIP /OP /AIP/DAD /DAH • Common in diffuse SSc – where the elbows and knees are involved • Typical serological attributes • ANA • Special • Nucleolar pattern, anti centromere pattern • Early diagnosis and treatment • Older male patients, antibody positive African American • Early decline in FVC
- 26. AIM (AUTO IMMUNE MYOSITIS) / IDIOPATHIC MYOPATHY • Historical / Physical • Idiopathic inflammatory • Dysphagia, proximal muscle weakness, photosensitive, Pulmonary arterial H’Tn • Common Imaging findings • Respiratory muscle dysfunction, aspiration • NSIP & OP followed by UIP and AIP • AIP/DAD Pneumo-mediastinum • Poor prognosis in older age groups • Concomitant PAH is prognostic indicator • Typical serological attributes • Creatinine kinase, aldolase • Special • Skin nodules, dermatomyositis, and ILD • PAH • Commonest in • SSc • Mixed conncective tissue disease • Less common • SLE • RA - Rare
- 27. SYSTEMIC LUPUS ERYTHEMATOSUS – ALVEOLAR HEMORRHAGE • Historical / Physical • Joint pain, alopecia, oral ulcers, pleurisy, rash, photosensitivity, pericarditis • Common Imaging findings • Acute pneumonitis • Alveolar hemorrhage • NSIP/UIP • DAH • OP • Typical serological attributes
- 28. DIAGNOSTIC CLUE - PULMONARY DISEASE SUSPECTED CASE • Historical and Examination • ILD with minimal phenotype or serological suggestive but not definitive for CVD • IPAF – Interstitial pneumonia with autoimmune features • Phenotypically / Radiology guided cost effective serological screen • Wider serological test profile in unclear symptomatic / imaging profile Silva CIS, Radiol Bras. 2008;41(3):189–197.
- 29. DRUG INDUCED LUNG DISEASE • DMRD and Biological agents • Rheumatoid arthritis • Diagnosis of exclusion / discontinued • Methotrexate – Acute pneumonitis • Sulfasalazine / Gold – obliterative bronchiolitis • Tumor necrotic factores – worsening ILD
- 30. MIXED CONNECTIVE TISSUE DISEASE • Historical / Physical • Variable signs & symptoms primarily associated with SLE, SSc, and AIM • Common Imaging findings • NSIP • LIP • Typical serological attributes • ANA • Special • Nuclear speckled pattern
- 31. HRCT PREDICTORS OF PROGRESSIVE FA IN CVD • FA – Fibrosing alveolitis • Progression > 15 % TLCO • Increased GGO • Increased reticular pattern • Bi-basal crackles • Exudative / Reparative phase • carbon monoxide transfer factor (TLCO)
- 32. SSC – HRCT SCORING FOR EVALUATING ILD • HRCT gold standard for SSc – ILD • 20 Yrs. Previously used to assess IPF • Wells et. al. Parenchymal and reticular • Morelli et. al. 3 zones • Apices to carina • Carina to inferior pulmonary veinous confluence • Pulmonary vein to diaphragm • Parenchymal opacification 1 • Parenchymal opacification > reticular pattern extent 2 • Parenchymal opacification = reticular pattern extent 3 Anatomical region scored • Reticular pattern > parenchymal opacification 4 • Reticular patter alone 5
- 33. SCREENING FOR ILD – INFLAMMATORY ARTHRITIS • Early referral • Symptoms (dyspnea, cough) • Physical examination findings (ambulatory hypoxemia) • Clubbing, Bibasilar crackles • Decrements on baseline PFTs • restrictive lung disease or diminished 6-min walk distance • ILD seen on HRCT • ILD with associated Arthritis • Cross referral to rheumatologist on suspicion
- 34. RAPIDLY PROGRESSIVE ILD - HOSPITALIZED PATIENT • Unexplained progression / rapidly progressive respiratory disease • SSc Spectrum • Sclerodactaly • Limited oral aperture • Nail fold capillary changes • Telangectsia • Adult onset reynaud’s • Dermatomyositis or anti-synthetase syndrome (both) • Skin rashes on extremities or face (both) • Nodules, diffuse erythema, plaques on the extensor surfaces • Periungual erythema, nail fold capillary microscopic changes • “mechanics hands,” which are fissures and cracking seen on the tips and radial aspect of the fingers
- 35. RAPIDLY PROGRESSIVE ILD - HOSPITALIZED PATIENT • RA • Symmetrical inflammatory arthritis in the hands and feet • SLE • skin rash, livedo reticularis, and joint pain, • young female patients • Vasculitis ANCA – (Antineutrophil cytoplasmic antibody) associated disease • sinusitis with or without epistaxis, otitis, nasal septal perforation • palpable purpura, and mononeuritis multiplex
- 36. SPECIFIC SEROLOGICAL TEST – HRCT IN UNCLEAR PHENOTYPE • Definite UIP • Rheumatoid Factor, CCP – anti cyclic citrullinated peptide, ANA, ANCA • NSIP, OP, Undefined or AIP • Anti nuclear antibody – ANA, RF, anti CCP, myositis anti body panel • creatinine kinase, aldolase, Melanoma differentiatin associated protein MDA5 • Bronchiolitis (obliterative bronchiolitis) • RF, anti-CCP • Diffuse alveolar hemorrhage • ANCA, ANA, antiphospholipid antibody, cryo globulins, anti glomerular basement membrane antibody • LIP ANA, RF, anti-CCP
- 37. ILD IN ARTHRITIS APPROACH TO SCREENING • Consensus based • Early referral to pulmonary medicine • Dyspnea / cough • Physical examination • Ambulatory hypoxemia, clubbing, bibasilar crackles • Decrimental baseline PFT • Restrictive lung disease, diminished 6 minute walking distance • ILD seen on HRCT • Warrants detailed rheumatological evaluation
- 38. SYSTEMIC SCLEROSIS • Early evidence of ILD and PAH is standard care • Screening baseline PFT – Spirometery, TLC, Diffusion Capacity • Echocardiography • HRCT • Normal is encouraging, abnormal baseline scan variable progression • Follow up with > 20% fibrotic lung • Follow up annual PFT • >10 % FVC or > 15 % DLCO • 6 minute walking test not validated in SSc can be useful in follow up evaluation. • Oesophageal dysmotility and aspiration risk.
- 39. AIM – AUTOIMMUNE MYOSITIS • Anti-synthetase syndrome • Screening • HRCT and PFT • HRCT abnormal and patient is symptomatic with < 70 % FVC treated • PFT biannually at 3 to 6 months • Oesophageal dysmotility. • PAH
- 40. RHEUMATOID ARTHRITIS • HRCT in high risk – older male smoker • PFT 3 to 6 months if the HRCT is abnormal • Decline of 10 % in PFT in an RA – ILD higher mortality risk • CT scanning for cancer screening • Subclinical ILD
- 41. PRESENT AND FUTURE • Predominantly anti inflammatory • Emerging agents targeting fibrosis • Cellular NSIP & OP • Cyclophosphamide, AZA, Calcineurin inhibitor • Fibrosing NSIP or UIP • Pirfenidone, Nintedanib • ILD with Lymphoid LIP in RA or Sjogrens syndrome • B Cell deletion – Rituximab
- 42. IN SUMMARY 20 11 3 7 5 29 75 0 10 20 30 40 50 60 70 80 RA SLE Sjogren's SSc Polymyositis Scleroderma Total Total Bronchiectasis Lymphadenopathy
- 43. IN SUMMARY 0 0.5 1 1.5 2 2.5 3 3.5 Rheumatoid Arthritis Progressive systemic Dermatopolymyositis Sjogren's syndrome Mixed connective tissue Systemic lupus UIP NSIP OP LIP DAD Hemorrhage Airway Disease
- 44. IN SUMMARY Intersection between pulmonary and rheumatologic disease represents a unique and often ambiguous paradigm of medicine requires vigilance and meticulous assessment but most importantly a multidisciplinary approach involving • Pulmonary medicine • Rheumatology • Radiology • Pathology • Thoracic surgery and other disciplines. Greater recognition and understanding of these disorders, Improved diagnostic and screening strategies, and more targeted therapies Outcomes and quality of life for this group of challenging patients will
- 45. THANK YOU FOR YOUR KIND ATTENTION Facebook – Medical Imaging Portal
Editor's Notes
- Systemic sclerosis – SSc / Scleroderma Commonest upto 38% Auto immune myositis- AIM Rheumatoid arthritis – RA upto 60% RA-ILD Systemic lupus erythematosus – SLE 14.6% o Sjogrens syndrome least common with 4%
- air-containing cystic spaces having thick, fibrous walls lined by bronchiolar epithelium
- Typical Ground-glass opacities, mild reticulation,traction bronchiectasis and bronchiolec-tasis, absent honeycombing in the earlystages Typical Distribution Ground-glass opacities, mild reticulation,traction bronchiectasis and bronchiolec-tasis, absent honeycombing in the earlystages Unusual Finding Extensive reticular pattern, consolida-tion (may represent areas of co-exist-ing organizing pneumonia – BOOP),peribronchovascular distribution
- Diffuse Alveolar Damage Typical findings Ground-glass opacity, “crazy paving” pattern, bilateral areas of consolidation maybe present mainly in dependent regions Typical distribution Bilateral and diffuse, rarely multifocal Unusual finding May progress to pulmonary fibrosis. Patients who survive may have HRCT pattern similar to non-specific interstitial pneumonia
- Typical findings Reticular pattern, bronchiectasis andtraction bronchiectasis, honeycombing,absent or minimal ground-glass opacities Typical distribution Peripheral, basal and posterior pre-dominance Unusual finding Asymmetrical distribution, major in-volvement of upper lobes
- Typical finding Consolidation is the typical finding,ground-glass opacity usually is associatedwith areas of consolidation, perilobularpattern or reversed halo sign may befound Typical distribution Bilateral symmetrical or asymmetrical,peripheral and/or peribronchovascularin 60–80% of cases, predominance inlower lobes Unusual finding Focal consolidation mimicking massmay progress to fibrosis
- Cryptogenic organizing pneumonia in a 69-year-old woman with polymyositis. Axial high-resolution CT image of the left lung shows consolidation in the lower lobe with multiple occurrences of the reverse halo sign (arrows), a feature characterized by a ring-shaped opacity surrounding a region of ground-glass opacity. MDA5 - Autoantibodies against melanoma differentiation-associated protein 5
- Typical finding Ground-glass opacities associated withsparse cysts ranging from 1–3 cm in di-ameter Typical distribution May be diffuse or predominate in thelower lobes; cysts may have subpleu-ral and peripheral distribution Unusual finding Isolated pulmonary cysts, ground-glassopacity as an isolated finding, septalthickening, and centrolobular nodules
- CAPLANS SYNDROME
- Shrinking lung syndrome, defined as a progressive loss of lung volume with associated dyspnea and pleuritic chest pain, was first described in 1965 (53).This is an uncommon manifestation of systemic lupus erythematosus, with a prevalence of only 0.6%
- systemic disorder characterized by excessive fibroblast proliferation, autoimmunity, and endothelial dysfunction leading to organ dysfunction involving the skin, lung, heart, and kidneys. Pulmonary involvement in ankylosing spondylitis in a 44-year-old man. Coronal high-resolution CT image shows apical reticular opacities with fibrosis, and a cavity containing an aspergilloma in the left lung.
- Pulmonary hypertension in a 27-year-old woman with progressive systemic sclerosis. Unenhanced axial CT image depicts enlargement of the pulmonary artery(PA)trunk and a substantial amount of pericardial effusion (arrows) in the anterior superior aortic recess. Historical / Physical Idiopathic inflammatory Dysphagia, proximal muscle weakness, photosensitive rash on hands/chest/face/back ,mechanics hands, anti synthetase syndrome (Raynaud syndrome, mechanics hands, fever, arthritis, ILD) Common Imaging findings morbidity and mortality relates to associated lung disease, including respiratory muscle dysfunction, aspiration, and ILD, most notably NSIP and OP followed by UIP and AIP NSIP OP UIP AIP/DAD Pneumo mediastinum Poor prognosis in worse prognosis is noted in older age groups, acute and subacute presentations with low initial FVC, amyopathic cases, and when concomitant PAH is present Typical serological attributes Creatinine kinase, aldolase Special Skin nodules, dermatomyositis, and ILD Historical / Physical Idiopathic inflammatory Dysphagia, proximal muscle weakness, photosensitive rash on hands/chest/face/back ,mechanics hands, antisynthetase syndrome (Raynaud syndrome, mechanics hands, fever, arthritis, ILD) Common Imaging findings morbidity and mortality relates to associated lung disease, including respiratory muscle dysfunction, aspiration, and ILD, most notably NSIP and OP followed by UIP and AIP NSIP OP UIP AIP/DAD Pneumomediastinum Poor prognosis in worse prognosis is noted in older age groups, acute and subacute presentations with low initial FVC, amyopathic cases, and when concomitant PAH is present Typical serological attributes Creatinine kinase, aldolase Special Skin nodules, dermatomyositis, and ILD
- Diffuse alveolar hemorrhage in a patient with systemic lupus erythematosus. Axial high-resolution CT image obtained in a 34-year-old woman with dyspnea, hemoptysis, and a low serum hemoglobin level shows bilateral central ground-glass opacities. The combined clinical and imaging findings are indicative of diffuse alveolar hemorrhage.
- NSIP in a 52-year-old womanwith mixed connective tissue disease. Axialhigh-resolution CT image demonstrates re-ticular abnormalities and peripheral ground-glass opacities in the lower lung lobes (ar-rows) with relative subpleural sparing.
- carbon monoxide transfer factor (TLCO) Predictors of progression of HRCTdiagnosed fibrosing alveolitis inpatients with rheumatoid arthritis J K Dawson1,H E Fewins2,J Desmond3,M P Lynch1,D R Graham4 BMJ Volume61,Issue6
- systemic disorder characterized by excessivefibroblast proliferation, autoimmunity, and endothelialdysfunction leading to organ dysfunction involving theskin, lung, heart, and kidneys.
- systemic disorder characterized by excessive fibroblast proliferation, autoimmunity, and endothelial dysfunction leading to organ dysfunction involving the skin, lung, heart, and kidneys.