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Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
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Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
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Scleroderma

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Case records of SGTH: Scleroderma with nephrotic syndrome.

Case records of SGTH: Scleroderma with nephrotic syndrome.

Published in: Health & Medicine, Education
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  • 1. Pulmonary & renal involvement in scleroderma Dr. Rauf R. Merza ( ASS.PROFESSOR ) Prepared by : Dr. Yahya A. Mohammad
  • 2. A 44 years old female from Kalar presented with swelling of both hands & feet of one month duration .
  • 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. <ul><li>swelling of both hands & feet . </li></ul><ul><li>associated with stiffness of hands without pain . </li></ul>
  • 5. <ul><li>her condition accompanied by bluish discoloration of hands & feet on exposure to cold . </li></ul>
  • 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. fever with rigor & sweating associated with exertional dyspnea .
  • 8. Systemic review : G.I.T. : dysphagia ( difficulty in swallowing solid food eased by drinking water ) , with
  • 9. Inability to open her mouth widely , heartburn , wt. loss , & constipation .
  • 10. C.V.S. : normal . Resp.S. : dry cough , exertional dyspnea . G.U.T. : bilateral loin pain , dysuria , &
  • 11. frequency . C.N.S. : normal . M.S.K. : generalized bodyache & fatigue .
  • 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. <ul><li>by development of Raynauds phenomena & gradual thickining of skin . </li></ul><ul><li>her disease continue </li></ul>
  • 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. <ul><li>Hx of HT since many years . </li></ul><ul><li>Past surgical Hx. : -ve </li></ul>
  • 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. Gyneocological Hx. : G6 P6 A0 Family Hx. : -ve Social Hx. : not smoker or drinker .
  • 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. <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. <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>
  • 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>
  • 24. end inspiratory crackles . Abdomen : soft .no organomegaly .
  • 25. Skin : Thickining of skin of hands , forearms , arms , neck , chest , & lower limbs .
  • 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>
  • 27. over the palmar surface of hands & flexion contractures of fingers specially little finger .
  • 28.  
  • 29.  
  • 30.  
  • 31.  
  • 32.  
  • 33. <ul><li>swelling of both feet </li></ul>
  • 34. Investigations
  • 35.  
  • 36.  
  • 37.  
  • 38.  
  • 39.  
  • 40.  
  • 41.  
  • 42.  
  • 43. Pulmonary Function Test
  • 44. restrictive lung dis. (pulm. fibrosis )
  • 45. CT scan of chest
  • 46.  
  • 47.  
  • 48.  
  • 49.  
  • 50.  
  • 51.  
  • 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>
  • 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>
  • 54. Scleroderma Classification : 1. Diffuse cutaneous SSc. 2. Limitted cut. SSc.
  • 55. 3. Overlap syndrome . 4. Undefined connective tissue dis. 5. Localized scleroderma .
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 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>
  • 66. THANK YOU

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