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Chronic Graft versus Host Disease Examples

Chronic Graft versus Host Disease Examples. May 28, 2012. Example # 1. Diane is a 36 year old On her clinic note you find the following: Maculopapular rash on her face and upper chest (15% BSA) Food sensitivity, lichen planus-like oral changes on physical exam

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Chronic Graft versus Host Disease Examples

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  1. Chronic Graft versus Host Disease Examples May 28, 2012

  2. Example # 1 Diane is a 36 year old On her clinic note you find the following: • Maculopapular rash on her face and upper chest (15% BSA) • Food sensitivity, lichen planus-like oral changes on physical exam • Dry eyes; using eye drops twice a day

  3. Example # 1 Does Diane have any diagnostic features of chronic graft versus host disease? Work through possible onset of chronic GVHD worksheet…

  4. Assessing Skin according to NIH Guidelines Diagnositic: • Poikiloderma • Lichen planus-like features • Sclerotic features • Morphea-like features Distinctive: • Depigmentation Common: • Erythema (erythroderma) • Pruritis • Maculopapular rash Additional features and/or reported symptoms: • Ichthyosis • Keratosis pilaris • Hyperpigmentation • Hypopigmentation • Papulsquamous lesions • Dry skin • Limited mobility

  5. Assessing Skin according to NIH Guidelines Diagnositic: • Poikiloderma • Lichen planus-like features • Sclerotic features • Morphea-like features Distinctive: • Depigmentation Common: • Erythema (erythroderma) • Pruritis x Maculopapular rash Additional features and/or reported symptoms: • Ichthyosis • Keratosis pilaris • Hyperpigmentation • Hypopigmentation • Papulsquamous lesions • Dry skin • Limited mobility

  6. Assessing Mouth according to NIH Guidelines Diagnostic: • Lichen-type features • Hyperkeratotic plaques • Restriction of mouth opening from sclerosis Distinctive: • Xerostomia (dry mouth) • Mucocele • Mucosal atrophy • Pseudomembranes • Ulcers Common: • Gingivitis • Mucositis • Erythema • Pain Additional features and/or reported symptoms: • Chapped lips • Bleeding gums • Sensitivity to spicy foods, toothpaste, etc

  7. Assessing Mouth according to NIH Guidelines Diagnostic: x Lichen-type features • Hyperkeratotic plaques • Restriction of mouth opening from sclerosis Distinctive: • Xerostomia (dry mouth) • Mucocele • Mucosal atrophy • Pseudomembranes • Ulcers Common: • Gingivitis • Mucositis • Erythema • Pain Additional features and/or reported symptoms: • Chapped lips • Bleeding gums x Sensitivity to spicy foods, toothpaste, etc

  8. Assessing Eyes According to the NIH Guidelines Diagnostic: (None) Distinctive: • New onset dry, gritty, or painful eyes • Cicatricial conjunctivitis • Keratoconjunctivitis sicca • Confluent areas of punctuate keratopathy Common: (none) Additional features and/or reported symptoms: • Photophobia • Periorbital hyperpigmentation • Blepharitis • Itchy eyes • Difficulty opening eyes in the morning • Excessive tearing • Diminished visual acuity and/or blurring

  9. Assessing Eyes According to the NIH Guidelines Diagnostic: (None) Distinctive: x New onset dry, gritty, or painful eyes • Cicatricial conjunctivitis • Keratoconjunctivitis sicca • Confluent areas of punctuate keratopathy Common: (none) Additional features and/or reported symptoms: • Photophobia • Periorbital hyperpigmentation • Blepharitis • Itchy eyes • Difficulty opening eyes in the morning • Excessive tearing • Diminished visual acuity and/or blurring

  10. Example # 1 Does Diane have Graft versus Host disease Yes but… Only the lichenoid changes in her mouth are diagnostic of graft versus host disease Once you have determined that the patient has diagnostic features of graft versus host disease then complete cGVHD scoring worksheet

  11. Scoring Skin cGVHD • 0 No Symptoms • 1 <18% BSA with disease signs but NO sclerotic features • 2 19-50% BSA OR involvement with superficial sclerotic features “not hidebound” (able to pinch) • 3 >50% BSA OR deep sclerotic features “hidebound” (unable to pinch) OR impaired mobility, ulceration or severe pruritus Calculating BSA and Rule of 9’s Head / Neck 9 % Left upper extremity 4.5 % front; 4.5 % back Right upper extremity 4.5 % front; 4.5 % back Anterior torso 18 % Posterior torso 18 % Left lower extremity 9 % front; 9 % back Right lower extremity 9 % front; 9 % back Genitalia 1 %

  12. Scoring Skin cGVHD • 0 No Symptoms X1 <18% BSA with disease signs but NO sclerotic features • 2 19-50% BSA OR involvement with superficial sclerotic features “not hidebound” (able to pinch) • 3 >50% BSA OR deep sclerotic features “hidebound” (unable to pinch) OR impaired mobility, ulceration or severe pruritus Calculating BSA and Rule of 9’s Head / Neck 9 % Left upper extremity 4.5 % front; 4.5 % back Right upper extremity 4.5 % front; 4.5 % back Anterior torso 18 % Posterior torso 18 % Left lower extremity 9 % front; 9 % back Right lower extremity 9 % front; 9 % back Genitalia 1 %

  13. Scoring the Mouth according to NIH Guidelines • 0 No Symptoms • 1 Mild symptoms with disease signs but not limiting oral intake significantly • 2 Moderate symptoms with disease signs with partial limitation of oral intake • 3 >Severe symptoms with disease signs on examination with major limitations of oral intake

  14. Scoring the Mouth according to NIH Guidelines • 0 No Symptoms X 1 Mild symptoms with disease signs but not limiting oral intake significantly • 2 Moderate symptoms with disease signs with partial limitation of oral intake • 3 >Severe symptoms with disease signs on examination with major limitations of oral intake

  15. Scoring the Eyes according to NIH Guidelines • 0 No Symptoms • 1 Mild dry eye symptoms not affecting ADL (requiring drops <3 x per day) OR asymptomatic signs of keratoconjunctivits sicca • 2 Moderate dry eye symptoms affecting ADL (requiring drops >3 x per day or punctual plugs), WITHOUT vision impairment • 3 Severe dry eye symptoms; significantly affecting ADL (special eye are to relive pain) OR unable to work because of ocular symptoms OR loss of vision caused by keratoconjunctivitis sicca

  16. Scoring the Eyes according to NIH Guidelines • 0 No Symptoms X 1 Mild dry eye symptoms not affecting ADL (requiring drops <3 x per day) OR asymptomatic signs of keratoconjunctivits sicca • 2 Moderate dry eye symptoms affecting ADL (requiring drops >3 x per day or punctual plugs), WITHOUT vision impairment • 3 Severe dry eye symptoms; significantly affecting ADL (special eye are to relive pain) OR unable to work because of ocular symptoms OR loss of vision caused by keratoconjunctivitis sicca

  17. Summary of Example # 1 Diane has the following GVHD scoring: Skin score – 1 Mouth score – 1 Eyes score -1

  18. Global Scoring of Chronic GVHD

  19. Chronic GVHD Global Score at time of this Assessment for Example # 1 • Mild • Moderate • Severe

  20. Chronic GVHD Global Score at time of this Assessment for Example # 1 • Mild X Moderate • Severe

  21. Example # 2 Julie is a 40 year old female Six month cGVHD assessment – July 27, 2011 • On June 10 2011 noted that she started 50mg of prednisone after seeing a respirologist for an FEV1 of 45% and FEV/VC predicted and severe SOB on any exertion • Respirologist opinion: bronchiolitis obliterans with interstitial lung disease post stem cell transplant secondary to graft versus host disease • She has no other features of cGVHD in any other organ

  22. Example # 2 Does Julie have any diagnostic or distinctive features of chronic graft versus host disease?

  23. Assessing Lungs According to NIH Guidelines Diagnostic: • Bronchiolitis obliterans diagnosed with lung biopsy Distinctive: • Bronchiolitis obliterans diagnosed with PFTs & radiology The following criteria must be present regarding BO: • FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of predicted. • Evidence of air trapping or small airway thickening or bronchiectasis on high-resolution chest CT scan (with inspiratory and expiratory cuts) residual volume >120%, or pathologic confirmation of constrictive bronchiolitis. • Absence of infection in the respiratory tract, documented with investigations directed by clinical symptoms, such as radiologic studies (radiographs or chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen, sputum culture, or bronchalveolar lavage). Common: • Bronchiolitis obliterans organizing pneumonia (BOOP) Additional features and/or reported symptoms: • Difficulty breathing • Wheezing • SOB at rest and/or exertion • Dry cough

  24. Assessing Lungs According to NIH Guidelines Diagnostic: • Bronchiolitis obliterans diagnosed with lung biopsy Distinctive: • Bronchiolitis obliterans diagnosed with PFTs & radiology The following criteria must be present regarding BO: X FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of predicted. • Evidence of air trapping or small airway thickening or bronchiectasis on high-resolution chest CT scan (with inspiratory and expiratory cuts) residual volume >120%, or pathologic confirmation of constrictive bronchiolitis. • Absence of infection in the respiratory tract, documented with investigations directed by clinical symptoms, such as radiologic studies (radiographs or chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen, sputum culture, or bronchalveolar lavage). Common: • Bronchiolitis obliterans organizing pneumonia (BOOP) Additional features and/or reported symptoms: • Difficulty breathing • Wheezing • SOB at rest and/or exertion • Dry cough

  25. Example # 2 Issues: • CT with inspiratory and expiratory cuts not done • Lung biopsy not done • No other diagnostic features of chronic graft versus host disease Therefore diagnosis of chronic graft versus host disease can not be made.

  26. Example # 2 Continues 1 year assessment: Lung: FEV1 – 83%; SOB on walking on flat ground Eyes: dry and gritty; Needs drops about 3-5 times per day she can’t read a book Mouth: Xerostomia; Erythema through the oral mucosa; she feels like mouth has glue in it and has taste disturbance with most foods Liver: AST -92 (> 2 x ULN); ALT 109 (< 2 x ULN)

  27. Assessing Lungs According to NIH Guidelines Diagnostic: • Bronchiolitis obliterans diagnosed with lung biopsy Distinctive: • Bronchiolitis obliterans diagnosed with PFTs & radiology The following criteria must be present regarding BO: • FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of predicted. • Evidence of air trapping or small airway thickening or bronchiectasis on high-resolution chest CT scan (with inspiratory and expiratory cuts) residual volume >120%, or pathologic confirmation of constrictive bronchiolitis. • Absence of infection in the respiratory tract, documented with investigations directed by clinical symptoms, such as radiologic studies (radiographs or chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen, sputum culture, or bronchalveolar lavage). Common: • Bronchiolitis obliterans organizing pneumonia (BOOP) Additional features and/or reported symptoms: • Difficulty breathing • Wheezing • SOB at rest and/or exertion • Dry cough

  28. Assessing Lungs According to NIH Guidelines Diagnostic: • Bronchiolitis obliterans diagnosed with lung biopsy Distinctive: • Bronchiolitis obliterans diagnosed with PFTs & radiology The following criteria must be present regarding BO: • FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of predicted. • Evidence of air trapping or small airway thickening or bronchiectasis on high-resolution chest CT scan (with inspiratory and expiratory cuts) residual volume >120%, or pathologic confirmation of constrictive bronchiolitis. • Absence of infection in the respiratory tract, documented with investigations directed by clinical symptoms, such as radiologic studies (radiographs or chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen, sputum culture, or bronchalveolar lavage). Common: • Bronchiolitis obliterans organizing pneumonia (BOOP) Additional features and/or reported symptoms: • Difficulty breathing • Wheezing X SOB at rest and/or exertion • Dry cough

  29. Assessing Eyes According to the NIH Guidelines Diagnostic: (None) Distinctive: • New onset dry, gritty, or painful eyes • Cicatricial conjunctivitis • Keratoconjunctivitis sicca • Confluent areas of punctuate keratopathy Common: (none) Additional features and/or reported symptoms: • Photophobia • Periorbital hyperpigmentation • Blepharitis • Itchy eyes • Difficulty opening eyes in the morning • Excessive tearing • Diminished visual acuity and/or blurring

  30. Assessing Eyes According to the NIH Guidelines Diagnostic: (None) Distinctive: XNew onset dry, gritty, or painful eyes • Cicatricial conjunctivitis • Keratoconjunctivitis sicca • Confluent areas of punctuate keratopathy Common: (none) Additional features and/or reported symptoms: • Photophobia • Periorbital hyperpigmentation • Blepharitis • Itchy eyes • Difficulty opening eyes in the morning • Excessive tearing • Diminished visual acuity and/or blurring

  31. Assessing Mouth according to NIH Guidelines Diagnostic: • Lichen-type features • Hyperkeratotic plaques • Restriction of mouth opening from sclerosis Distinctive: • Xerostomia (dry mouth) • Mucocele • Mucosal atrophy • Pseudomembranes • Ulcers Common: • Gingivitis • Mucositis • Erythema • Pain Additional features and/or reported symptoms: • Chapped lips • Bleeding gums • Sensitivity to spicy foods, toothpaste, etc

  32. Assessing Mouth according to NIH Guidelines Diagnostic: • Lichen-type features • Hyperkeratotic plaques • Restriction of mouth opening from sclerosis Distinctive: X Xerostomia (dry mouth) • Mucocele • Mucosal atrophy • Pseudomembranes • Ulcers Common: • Gingivitis • Mucositis XErythema • Pain Additional features and/or reported symptoms: • Chapped lips • Bleeding gums X Sensitivity to spicy foods, toothpaste, etc

  33. Assessing the Liver according to NIH Guidelines Diagnostic: (none) Distinctive: (none) Common: • Total bilirubin, alk phos >2x ULN plus ALT or AST>2x ULN

  34. Assessing the Liver according to NIH Guidelines Diagnostic: (none) Distinctive: (none) Common: X Total bilirubin, alk phos >2x ULN plus ALT or AST>2x ULN

  35. Example # 2 continues Julie has some distinctive features of cGVHD but no diagnostic features Go back and review with primary physician

  36. Further review with physician shows… She does have lichen-type features in her mouth… Therefore, a diagnosis of chronic GVHD can be made

  37. Scoring the Lungs according to NIH Guidelines • 0 No symptoms and/or FEV1> 80% OR LFS 3-5 • 1 Mild symptoms (SOB after climbing one flight of stairs) • 2 Moderate symptoms (shortness of breath after walking on flat ground) and/or FEV1 40-59% • 3 Severe symptoms (shortness of breath at rest requiring oxygen)

  38. Scoring the Lungs according to NIH Guidelines • 0 No symptoms and/or FEV1> 80% OR LFS 3-5 • 1 Mild symptoms (SOB after climbing one flight of stairs) X 2 Moderate symptoms (shortness of breath after walking on flat ground) and/or FEV1 40-59% • 3 Severe symptoms (shortness of breath at rest; requiring oxygen)

  39. Scoring the Eyes according to NIH Guidelines • 0 No Symptoms • 1 Mild dry eye symptoms not affecting ADL (requiring drops < 3 x per day) OR asymptomatic signs of keratoconjunctivits sicca • 2 Moderate dry eye symptoms affecting ADL (requiring drops >3 x per day or punctual plugs), WITHOUT vision impairment • 3 Severe dry eye symptoms; significantly affecting ADL (special eye are to relive pain) OR unable to work because of ocular symptoms OR loss of vision caused by keratoconjunctivitis sicca

  40. Scoring the Eyes according to NIH Guidelines • 0 No Symptoms • 1 Mild dry eye symptoms not affecting ADL (requiring drops < 3 x per day) OR asymptomatic signs of keratoconjunctivits sicca X 2 Moderate dry eye symptoms affecting ADL (requiring drops >3 x per day or punctual plugs), WITHOUT vision impairment • 3 Severe dry eye symptoms; significantly affecting ADL (special eye are to relive pain) OR unable to work because of ocular symptoms OR loss of vision caused by keratoconjunctivitis sicca

  41. Scoring the Mouth according to NIH Guidelines • 0 No Symptoms • 1 Mild symptoms with disease signs but not limiting oral intake significantly • 2 Moderate symptoms with disease signs with partial limitation of oral intake • 3 Severe symptoms with disease signs on examination with major limitations of oral intake

  42. Scoring the Mouth according to NIH Guidelines • 0 No Symptoms • 1 Mild symptoms with disease signs but not limiting oral intake significantly X 2 Moderate symptoms with disease signs with partial limitation of oral intake • 3 Severe symptoms with disease signs on examination with major limitations of oral intake

  43. Scoring the Liver according to NIH Guidelines • 0 Normal LFTs • 1 One or more of the following elevated > 2 X ULN: Total Bilirubin, Alk Phos, AST, ALT • 2 TotalBilirubin > 3 mg/dl (i.e. > 51 µmol/L) OR One or more of the following elevated 2-5 x ULN: Total Bilirubin, Alk Phos, AST, ALT • 3 One or more of the following elevated > 5 x ULN: Total Bilirubin, Alk Phos, AST, ALT

  44. Scoring the Liver according to NIH Guidelines • 0 Normal LFTs X 1 One or more of the following elevated > 2 X ULN: Total Bilirubin, Alk Phos, AST, ALT • 2 TotalBilirubin > 3 mg/dl (i.e. > 51 µmol/L) OR One or more of the following elevated 2-5 x ULN: Total Bilirubin, Alk Phos, AST, ALT • 3 One or more of the following elevated > 5 x ULN: Total Bilirubin, Alk Phos, AST, ALT

  45. Summary of Example # 2 Julie has the following GVHD scoring: Lung score – 2 Eye score – 2 Mouth score – 2 Liver Score - 1

  46. Global Scoring of Chronic GVHD

  47. Chronic GVHD Global Score at time of this Assessment for Example # 2 • Mild • Moderate • Severe

  48. Chronic GVHD Global Score at time of this Assessment for Example # 2 • Mild • Moderate X Severe

  49. Example # 3 Mark is a 49 year old, 110 days post stem cell transplant On his clinic note you find: He has lichen type changes in his mouth; no taste disturbance Just admitted to hospital in the last 2 days for diarrhea 1.5 litres a day with nausea and vomiting. He has lost 10kg and weighed 85kg at his last visit.

  50. Assessing Mouth according to NIH Guidelines Diagnostic: • Lichen-type features • Hyperkeratotic plaques • Restriction of mouth opening from sclerosis Distinctive: • Xerostomia (dry mouth) • Mucocele • Mucosal atrophy • Pseudomembranes • Ulcers Common: • Gingivitis • Mucositis • Erythema • Pain Additional features and/or reported symptoms: • Chapped lips • Bleeding gums • Sensitivity to spicy foods, toothpaste, etc

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