Scleroderma

1,952 views
1,797 views

Published on

Case records of SGTH: Scleroderma with nephrotic syndrome.

Published in: Health & Medicine, Education
0 Comments
3 Likes
Statistics
Notes
  • Be the first to comment

No Downloads
Views
Total views
1,952
On SlideShare
0
From Embeds
0
Number of Embeds
9
Actions
Shares
0
Downloads
166
Comments
0
Likes
3
Embeds 0
No embeds

No notes for slide

Scleroderma

  1. 1. Pulmonary & renal involvement in scleroderma Dr. Rauf R. Merza ( ASS.PROFESSOR ) Prepared by : Dr. Yahya A. Mohammad
  2. 2. A 44 years old female from Kalar presented with swelling of both hands & feet of one month duration .
  3. 3. <ul><li>H.P.I. </li></ul><ul><li>a known case of scleroderma since 13 y. </li></ul><ul><li>her condition had been started as a </li></ul>
  4. 4. <ul><li>swelling of both hands & feet . </li></ul><ul><li>associated with stiffness of hands without pain . </li></ul>
  5. 5. <ul><li>her condition accompanied by bluish discoloration of hands & feet on exposure to cold . </li></ul>
  6. 6. <ul><li>in the last 3 weeks she develop an attack of dry cough which continue untill now with moderate to high grade intermittent </li></ul>
  7. 7. fever with rigor & sweating associated with exertional dyspnea .
  8. 8. Systemic review : G.I.T. : dysphagia ( difficulty in swallowing solid food eased by drinking water ) , with
  9. 9. Inability to open her mouth widely , heartburn , wt. loss , & constipation .
  10. 10. C.V.S. : normal . Resp.S. : dry cough , exertional dyspnea . G.U.T. : bilateral loin pain , dysuria , &
  11. 11. frequency . C.N.S. : normal . M.S.K. : generalized bodyache & fatigue .
  12. 12. <ul><li>Past medical Hx. : </li></ul><ul><li>a known case of scleroderma since 13 years . </li></ul><ul><li>her condition started </li></ul>
  13. 13. <ul><li>by development of Raynauds phenomena & gradual thickining of skin . </li></ul><ul><li>her disease continue </li></ul>
  14. 14. <ul><li>in relapse & remission in the last 13 years . </li></ul><ul><li>admitted to hospitals many times ( once every 2-3 months ) . </li></ul>
  15. 15. <ul><li>Hx of HT since many years . </li></ul><ul><li>Past surgical Hx. : -ve </li></ul>
  16. 16. <ul><li>Drug Hx. : on </li></ul><ul><li>captopril tab. 25mg 1*2 </li></ul><ul><li>amilodipine tab. 10mg 1*1 </li></ul>
  17. 17. Gyneocological Hx. : G6 P6 A0 Family Hx. : -ve Social Hx. : not smoker or drinker .
  18. 18. <ul><li>EXAMINATION </li></ul><ul><li>BP 180/100 mmhg. </li></ul><ul><li>PR 85/min. </li></ul><ul><li>RR 18/min. </li></ul><ul><li>Temp. 38.6 C </li></ul>
  19. 19. <ul><li>General exam. : </li></ul><ul><li>conscious , allert middle aged female . </li></ul><ul><li>depressed . </li></ul><ul><li>small mouth aperature </li></ul>
  20. 20. <ul><li>with puckering . </li></ul><ul><li>no pallor . Jaundice , lymphadenopathy , or clubbing of fingers . </li></ul>
  21. 22. <ul><li>Precordium : </li></ul><ul><li>normal S1 , S2 , no added sounds . </li></ul><ul><li>Chest : </li></ul><ul><li>bilateral chest expansion </li></ul>
  22. 23. <ul><li>decrease . </li></ul><ul><li>trachea centrally located . </li></ul><ul><li>percussion resonant. </li></ul><ul><li>auscultation , diffuse </li></ul>
  23. 24. end inspiratory crackles . Abdomen : soft .no organomegaly .
  24. 25. Skin : Thickining of skin of hands , forearms , arms , neck , chest , & lower limbs .
  25. 26. <ul><li>MSK. : </li></ul><ul><li>normal gait , cervical s. ,shoulders ,& elbow j. </li></ul><ul><li>swelling of both hands with telegectasia </li></ul>
  26. 27. over the palmar surface of hands & flexion contractures of fingers specially little finger .
  27. 33. <ul><li>swelling of both feet </li></ul>
  28. 34. Investigations
  29. 43. Pulmonary Function Test
  30. 44. restrictive lung dis. (pulm. fibrosis )
  31. 45. CT scan of chest
  32. 52. <ul><li>Treatment </li></ul><ul><li>ceftriaxone vial 1g 1*2 IV. </li></ul><ul><li>capoten tab. 50mg 1*3 </li></ul>
  33. 53. <ul><li>amilodipine tab. 10mg 1*1 </li></ul><ul><li>paracetamol amp. 1*3 </li></ul><ul><li>losec cap. 40mg 1*2 </li></ul>
  34. 54. Scleroderma Classification : 1. Diffuse cutaneous SSc. 2. Limitted cut. SSc.
  35. 55. 3. Overlap syndrome . 4. Undefined connective tissue dis. 5. Localized scleroderma .
  36. 56. <ul><li>Diffuse cutaneous SSc. </li></ul><ul><li>proximal skin thickening involving face/neck,trunk,& symmetrically the fingers , hands , arms , & legs. </li></ul><ul><li>rapid onset of dis. following Raynauds ph. </li></ul>
  37. 57. <ul><li>significant visceral dis. : lung , heart , gastrointestinal , or kidney . </li></ul><ul><li>positive ANA & negative anticetromere Ab . </li></ul><ul><li>poor prognosis ( survival 40%-60% at 10 years ) . </li></ul>
  38. 58. <ul><li>Pulmonary manifestations : </li></ul><ul><li>Pulmonary disease is now the leading cause of death in </li></ul><ul><li>SSc . </li></ul><ul><li>Patients with dcSSc are at </li></ul>
  39. 59. <ul><li>Higher risk of developing significant lung fibrosis compared to those with lcSSc. </li></ul><ul><li>may be asymptomatic or associated with dry cough or exertional dyspnea . </li></ul>
  40. 60. <ul><li>PFT that show a restrictive pattern is the most sensitive test for pulmonary parenchymal dis. </li></ul><ul><li>Pulmonary hypertension can occur . </li></ul>
  41. 61. <ul><li>Cyclophosphamide (CYC) has been used as the primary </li></ul><ul><li>therapeutic agent for interstitial lung disease in scleroderma. </li></ul>
  42. 62. <ul><li>Renal dis. & scleroderma renal crisis : </li></ul><ul><li>Scleroderma renal crisis (SRC) was the most common cause of death in SSc prior </li></ul>
  43. 63. <ul><li>to the introduction of angiotensin-converting enzyme (ACE) inhibitors . </li></ul><ul><li>Clinical signs and symptoms are severe hypertension and can include headaches, </li></ul><ul><li>stroke, and heart failure. </li></ul>
  44. 64. <ul><li>The creatinine is elevated & urinalysis shows proteinuria and microscopic hematuria. </li></ul><ul><li>Poor prognostic factors in SRC include a creatinine level >3 mg/dL at the time of diagnosis of SRC, delay in </li></ul>
  45. 65. <ul><li>blood pressure normalization >3 days, male sex, older age, and presence of congestive heart failure. </li></ul><ul><li>treated by ACE inhibitor & dialysis . </li></ul>
  46. 66. THANK YOU

×