A Case of Henoch-Schonlein Purpura

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  • A Case of Henoch-Schonlein Purpura

    1. 1. PROF.DR.NOORUL AMEEN DR.R.PANDICHELVAN M6
    2. 2. <ul><li>23 yrs old male patient admitted on 5.2.11 </li></ul><ul><li>in surgical ward with </li></ul><ul><li>H/o abdominal pain 14 days </li></ul><ul><li>more in right lower quadrant </li></ul><ul><li>colicky </li></ul><ul><li>intermittent pain </li></ul><ul><li>no radiation </li></ul><ul><li>not related with food intake </li></ul>
    3. 3. <ul><li>No h/o vomiting </li></ul><ul><li>No h/o fever </li></ul><ul><li>No h/o diarrhoea </li></ul><ul><li>No h/o jaundice </li></ul><ul><li>No h/o cough </li></ul><ul><li>No h/o dysuria </li></ul>
    4. 4. <ul><li>On examination pt Conscious </li></ul><ul><li>Oriented </li></ul><ul><li>Afebrile </li></ul><ul><li>A o /J o /Cy o /Cl o </li></ul><ul><li>CVS S1S2 </li></ul><ul><li>RS NVBS </li></ul><ul><li>P/A tenderness in right iliac fossa </li></ul><ul><li>CNS NFND </li></ul>
    5. 5. <ul><li>clinically diagnosed as </li></ul><ul><li>Acute appendicitis and treated with </li></ul><ul><li>NPO </li></ul><ul><li>IVF DNS and NS </li></ul><ul><li>INJ Ciprofloxin 200mg iv bd </li></ul><ul><li>INJ Metronidazole 500mg iv tds </li></ul><ul><li>INJ Ranitidine 50 mg iv bd </li></ul><ul><li>Planned for surgery </li></ul>
    6. 6. <ul><li>Patient developed skin rashes in both lower extremities and physician opinion was obtained for a/c appendicitis with ? drug allergy and fitness for surgery </li></ul>
    7. 7. <ul><li>General examination : </li></ul><ul><li>Pt Conscious </li></ul><ul><li>Oriented </li></ul><ul><li>Afebrile </li></ul><ul><li>I * P * CY * CL * LNE * </li></ul><ul><li>Purpura – both lower legs upto thigh and in both upper limbs -palpable ;confluent </li></ul><ul><li>Bp 110/80 mmHg </li></ul><ul><li>PR 86/min </li></ul><ul><li>CVS S1S2 </li></ul><ul><li>RS NVBS </li></ul><ul><li>CNS NFND </li></ul>
    8. 8. <ul><li>PICTURE </li></ul>
    9. 11. <ul><li>P/A Not distended </li></ul><ul><li>Moves with respiration </li></ul><ul><li>Flanks free </li></ul><ul><li>No distended veins </li></ul><ul><li>No visible peristalis </li></ul><ul><li>Soft </li></ul><ul><li>Tender </li></ul><ul><li>No organomegaly </li></ul><ul><li>No free fluid </li></ul><ul><li>BS heard </li></ul>
    10. 12. <ul><li>Past history: Patient admitted in GRH for the c/o arthralgia and skin rashes 10 days ago </li></ul><ul><li>diagnosed as post viral arthralgia ; treated and discharged </li></ul><ul><li>Not k/c/o DM; HT; PT </li></ul><ul><li>Personal history: </li></ul><ul><li>Not an alcoholic;smoker </li></ul><ul><li>Patient took over to medical ward </li></ul>
    11. 13. <ul><ul><li>Provisional diagnosis </li></ul></ul><ul><li>A/C abdominal pain with purpura for evaluation </li></ul>
    12. 14. <ul><li>Pt managed with </li></ul><ul><li>IVF DNS 2 pints </li></ul><ul><li>Inj.Ciprofloxin 200 mg iv bd </li></ul><ul><li>Inj.Metronidazole 500 mg iv tds </li></ul><ul><li>Inj.Ranitidine 50 mg iv bd </li></ul><ul><li>Tab.Paracetamol 500mg tds </li></ul>
    13. 15. <ul><li>Differential diagnosis for medical causes of pain abdomen </li></ul><ul><li>Diabetic ketoacidosis </li></ul><ul><li>Herpes zoster </li></ul><ul><li>Sickle cell anemia </li></ul><ul><li>A/c intermittent porphyria </li></ul><ul><li>Poly arteritis nodosa </li></ul><ul><li>Henoch schonelin purpura </li></ul><ul><li>Lead poisoning </li></ul><ul><li>Hyperlipidaemia </li></ul><ul><li>Allergic pain [C1 esterase inhibitor deficiency] </li></ul>
    14. 16. <ul><li>Differential diagnosis for palpable purpura </li></ul><ul><li>1. Vasculitis </li></ul><ul><li>a.Cutaneous small vessel vasculitis </li></ul><ul><li>B.Polyartritis nodosa </li></ul><ul><li>C.Essential mixed cryoglobulinemia </li></ul><ul><li>2.Emboli </li></ul><ul><li>A.A/c menigococcemia </li></ul><ul><li>B.Disseminated gonococcal infection </li></ul><ul><li>C.Rocky mountain spotted fever </li></ul><ul><li>D.Ecthyma gangrenosum </li></ul>
    15. 17. <ul><li>Urine Albumin- trace </li></ul><ul><li>Sugar - nil </li></ul><ul><li>Deposit- 1-2pc/hpf </li></ul><ul><li>CBC </li></ul><ul><li>HB- 12.2GM/DL </li></ul><ul><li>TC-6400 </li></ul><ul><li>DC – P 65; L 32; E 3 </li></ul><ul><li>ESR -8/12 </li></ul><ul><li>PLC 345000 </li></ul><ul><li>MCV 82.2 </li></ul><ul><li>MCH 24.5 </li></ul><ul><li>MCHC 31.2 </li></ul><ul><li>PCV 38 % </li></ul>
    16. 18. <ul><li>RFT : </li></ul><ul><li>Urea -14 </li></ul><ul><li>Creatine- 0.6 </li></ul><ul><li>RBS – 87 </li></ul><ul><li>Na -132 </li></ul><ul><li>K-3.7 </li></ul><ul><li>Cl -94 </li></ul><ul><li>Bicarbonate-18 </li></ul><ul><li>ECG : Normal </li></ul><ul><li>CXR : Normal </li></ul>
    17. 19. <ul><li>USG abdomen : Normal except for probe tenderness ; no appendicular mass </li></ul><ul><li>CT abdomen : Peri appendicular edema seen </li></ul><ul><li>suggestion: a/c appendicitis </li></ul>
    18. 20. <ul><li>SP 3 09.02.11 </li></ul><ul><li>CBC </li></ul><ul><li>Platelet count -424000 </li></ul><ul><li>TC -12800 cells/ cumm </li></ul><ul><li>RBC -5.51million/ul </li></ul><ul><li>DC – P78; L12; E10 </li></ul><ul><li>PCV -45.7% </li></ul><ul><li>MCV -32.9 </li></ul><ul><li>MCH - 26.9 </li></ul><ul><li>MCHC -32.4 </li></ul><ul><li>HB -14.8 </li></ul><ul><li>ESR -30mm/hr </li></ul><ul><li>Prothrombin time 14 sec </li></ul><ul><li>APTT 26 sec </li></ul><ul><li>INR 1.0 </li></ul>
    19. 21. <ul><li>BIOCHEMISTRY </li></ul><ul><li>Sugar -87 </li></ul><ul><li>Urea – 21.8 </li></ul><ul><li>Creatine – 0.74 </li></ul><ul><li>LIVER FUNCTION TEST </li></ul><ul><li>Total bilirubin -0.52 </li></ul><ul><li>Direct bilirubin -0.20 </li></ul><ul><li>AST -25 u/l </li></ul><ul><li>ALT -17u/l </li></ul><ul><li>GGT – 17u/l </li></ul><ul><li>SAP - 160u/l </li></ul><ul><li>Total protein -7g/dl </li></ul><ul><li>Albumin – 4g/dl </li></ul><ul><li>Globulin -2.9 g/dl </li></ul>
    20. 22. <ul><li>ELECTROLYTES: </li></ul><ul><li>Na -141 </li></ul><ul><li>K - 4.89 </li></ul><ul><li>Cl -103.4 </li></ul><ul><li>SEROLOGY </li></ul><ul><li>HBSAG – Negative </li></ul><ul><li>ANTIHCV- Negative </li></ul><ul><li>HIV - Negative </li></ul><ul><li>URINE MICROSCOPY </li></ul><ul><li>Leucocytes -3-4/hpf </li></ul><ul><li>Ep cells - occassional </li></ul><ul><li>RBc -2-3/hpf </li></ul><ul><li>SERUM LDH 219 u/l </li></ul>
    21. 23. <ul><li>Urine analysis 14.02.2011 </li></ul><ul><li>Glu -trace </li></ul><ul><li>Bil -neg </li></ul><ul><li>Ketone neg </li></ul><ul><li>Sg - 1.015 </li></ul><ul><li>Blo 2+ </li></ul><ul><li>ph 7.5 </li></ul><ul><li>Pro 3+ </li></ul><ul><li>Nit neg </li></ul><ul><li>Leu neg </li></ul><ul><li>STOOL Ova nil </li></ul><ul><li>Cyst nil </li></ul><ul><li>occult blood nil </li></ul>
    22. 24. <ul><li>IMMUNOLOGY </li></ul><ul><li>ANA 1:10 NEG </li></ul><ul><li>1:40 NEG </li></ul><ul><li>ASO NEG </li></ul><ul><li>RF NEG </li></ul><ul><li>CRP NEG </li></ul><ul><li>C ANCA NEG </li></ul><ul><li>P ANCA NEG </li></ul><ul><li>Ig A 391mg/dl [ normal 80-350 mg/dl] </li></ul>
    23. 25. <ul><li>Histopathology report </li></ul><ul><li>Specimen :purpura right forearm biopsy </li></ul><ul><li>Microscopic description :stratified squamous epithelium with hyperkeratosis , subepithelium shows congested blood vessels infiltrated with lymphocytes </li></ul><ul><li>Impression : cutaneous small vessel vasculitis </li></ul>
    24. 28. <ul><li>DIAGNOSIS </li></ul><ul><li>HENOCH SCHONLEIN PURPURA </li></ul><ul><li>Patient treated with Tab. Prednisolone 1mg/kg </li></ul><ul><li>Tab.Ranitidine 150 mg bd </li></ul><ul><li>Tab.BCT tds </li></ul>
    25. 29. <ul><li>HSP is characterized by a classic tetrad of </li></ul><ul><li>Non thrombocytopenic palpable purpura </li></ul><ul><li>Arthralgia or arthritis </li></ul><ul><li>Gastrointestinal and renal involvement </li></ul><ul><li>Rarely involvement of lungs , cns,genitourinary tract </li></ul><ul><li>Cutaneous involvement is most common presentation </li></ul><ul><li>Also called anaphylactoid purpura </li></ul>
    26. 30. EPIDEMIOLOGY <ul><li>90% of cases reported in children </li></ul><ul><ul><li>Peak in children aged 4-7 </li></ul></ul><ul><li>Male:Female (1.5:1) </li></ul><ul><li>50% follow a URI </li></ul><ul><li>Renal disease is more severe in adults </li></ul><ul><li>Seasonal variation ; peak incidence in spring </li></ul>
    27. 31. PATHOGENESIS <ul><li>Likely mechanism thought to be an immune-complex mediated disease with deposits in the glomerular capillaries, dermal capillaries and GI tract. </li></ul><ul><li>Mesangial deposits of IgA are the same as those seen in IgA nephropathy </li></ul>
    28. 32. PRECIPITATING ANTIGENS <ul><li>INFECTIONS </li></ul><ul><ul><li>URI </li></ul></ul><ul><ul><li>Measles </li></ul></ul><ul><ul><li>Rubella </li></ul></ul><ul><ul><li>Parvovirus B19 </li></ul></ul><ul><ul><li>Mycoplasma </li></ul></ul><ul><ul><li>Coxsackie virus </li></ul></ul><ul><ul><li>Toxocara </li></ul></ul><ul><ul><li>Amebiasis </li></ul></ul><ul><ul><li>Salmonella </li></ul></ul><ul><ul><li>C.difficile </li></ul></ul><ul><ul><li>H.pylori </li></ul></ul><ul><ul><li>Adenovirus </li></ul></ul><ul><ul><li>Legionella </li></ul></ul><ul><ul><li>Tuberculosis </li></ul></ul><ul><ul><li>Mumps </li></ul></ul><ul><ul><li>Streptococcus </li></ul></ul><ul><ul><li>Morganella morganii </li></ul></ul>
    29. 33. PRECIPITATING ANTIGENS <ul><li>Drugs </li></ul><ul><ul><li>Vancomycin </li></ul></ul><ul><ul><li>Streptokinase </li></ul></ul><ul><ul><li>Ranitidine </li></ul></ul><ul><ul><li>Cefuroxime </li></ul></ul><ul><ul><li>Diclofenac </li></ul></ul><ul><ul><li>Enalapril </li></ul></ul><ul><ul><li>Captopril </li></ul></ul>
    30. 34. PRECIPITATING ANTIGENS <ul><li>Other: </li></ul><ul><ul><li>Food hypersensitivity </li></ul></ul><ul><ul><li>Cold exposure </li></ul></ul><ul><ul><li>Autosomal recessive Chronic granulomatous disease </li></ul></ul><ul><ul><li>Myelodysplastic syndrome </li></ul></ul><ul><ul><li>Small cell lung cancer </li></ul></ul><ul><ul><li>Breast cancer </li></ul></ul>
    31. 36. CLINICAL FEATURES <ul><li>PALPABLE PURPURA: most commonly seen on lower extremities and buttocks, however can also been seen on the trunk and arms. </li></ul><ul><ul><li>Lesions begin as erythematous macules and progress to purpuric, non-blanching, nonpruritic lesions that may become confluent </li></ul></ul>
    32. 37. Gastrointestinal manifestations <ul><li>One third with HSP have GI features </li></ul><ul><li>Colicky pain, nausea, vomiting, GI bleed </li></ul><ul><li>50% have occult bleeding </li></ul><ul><li>Major GI bleed in 5% </li></ul><ul><li>Intussusception 2% </li></ul><ul><li>Abdominal pain: sub-mucosal and intramural extravasation of fluid and blood </li></ul><ul><li>Endoscopy: Erosive duodenitis </li></ul>
    33. 38. Renal manifestations <ul><li>Abnormalities of urinary sediment </li></ul><ul><li>Acute nephritis </li></ul><ul><li>Progressive renal disease </li></ul><ul><li>Glomerular crescent formation - fatal outcome </li></ul><ul><li>Mortality 4% </li></ul><ul><li>Nephropathy adults 85%; children 25% </li></ul><ul><li>Renal insufficiency 13% in adults, 0% in children </li></ul>
    34. 39. HSP in Children & Adults FEATURES CHILDREN ADULTS Arthritis/ Arthralgia Knees, ankles, elbows, shoulders Wrists, MCP, PIP, MTP Edema Scalp, Face, Arms, Hands, Feet Scrotal Absent Purpura Limbs (usually dependent areas) Limbs, torso Abdominal pain Present Present Renal involvement Uncommon Common Course Benign More fulminant Familial Rare Absent
    35. 40. DIAGNOSTIC EVALUATION <ul><li>May have mild leukocytosis </li></ul><ul><li>Normal platelet count </li></ul><ul><li>Occasionally eosinophilia </li></ul><ul><li>Normal serum complement levels </li></ul><ul><li>Elevated IgA in 50% cases </li></ul>
    36. 41. DIAGNOSIS <ul><li>Generally a clinical diagnosis </li></ul><ul><li>Skin Biopsy: can be helpful and used to confirm IgA and C3 deposits and leukocytoclastic vasculitis. </li></ul><ul><li>Renal Biopsy: not usually needed for diagnosis. Will show mesangial IgA deposits and segmental glomerulonephritis </li></ul>
    37. 42. <ul><li>DIAGNOSTIC CRITERIA </li></ul><ul><li>ACR criteria-1990 </li></ul><ul><li>three or more of the following cricteria </li></ul><ul><li>1.age 20 yrs or less at disease onset </li></ul><ul><li>2.palpable purpura </li></ul><ul><li>3.a/c abdominal pain with git bleeding </li></ul><ul><li>4.biopsy show granulocytes in the walls of small arterioles and venules in superficial layers of skin </li></ul>
    38. 43. <ul><li>EuLAR/PReS criteria -2006 </li></ul><ul><li>mandatory criterion </li></ul><ul><li>1. palpable purpura </li></ul><ul><li>plus at least one of the following criteria </li></ul><ul><li>1.diffuse abdominal pain </li></ul><ul><li>2.iga deposition in any biopsy </li></ul><ul><li>3.arthritis or arthralgia </li></ul><ul><li>4.renal involvement[hematuria and or proteinuria] </li></ul>
    39. 44. <ul><li>Chapell hill consensus group classification system </li></ul><ul><li>primarily use nonclinical criteria </li></ul><ul><li>require only presence of small vesel vasculitis with IGA deposition </li></ul>
    40. 45. Medications Indication Comments Acetaminophen, NSAIDs Mild rash, arthritis Oral steroids (1-2 mg/Kg) Severe rash, cutaneous edema, severe colicky abdominal pain, scrotal and testicular involvement These cannot prevent development of systemic involvement but can be helpful for symptomatic treatment. IV steroids (1-2 mg/Kg) Same as oral steroids, should be given if patient is not able to tolerate oral medications Same as oral steroids
    41. 46. High-dose IV pulse steroids Nephrotic range proteinuria Decreases ESRD progression (in some case series and reports) High-dose IV pulse steroids plus immunosuppression Rapidly progressive glomerulonephritis (RPGN), hemorrhagic involvement of lungs, brain Plasmapheresis and/or IV immunoglobulin therapy Refractory HSP to combination therapy (steroids and immunosuppression), massive hemorrhage in gastrointestinal or other organs Grade D recommendation, but evidence is growing with multiple case series and reports. This is used as the last resort to treat refractory HSP.
    42. 47. MANAGEMENT <ul><li>Usually self-limiting (1-6 weeks) </li></ul><ul><li>Steroids: </li></ul><ul><ul><li>may decrease tissue edema, may aid in arthralgias and some abdominal pain </li></ul></ul><ul><ul><li>Has not been shown to be beneficial in kidney disease or dermal manifestations </li></ul></ul><ul><ul><li>Does not lessen chance of recurrence </li></ul></ul><ul><ul><li>Does not shorten duration of disease </li></ul></ul>
    43. 48. <ul><li>Prognostic factors for HSP : .The worse prognostic factors: (i) Greater than 8 years of age  (ii) Greater number of relapses  (iii) Higher creatinine level at the onset  (iv) Proteinuria greater than 1g/day  (v) Hematuria, anemia at diagnosis  (vi) Development of hypertension  (vii) Membranoproliferative glomerulonephritis  (viii) Fever at presentation  (ix) Purpura above the waist  (x) Persistent purpura  (xi) Elevated sedimentation rate.  (xii) Elevated IgA concentration with reduced IgM ( concentration at the time of diagnosis.)    (xiii) Low factor XIII level  </li></ul>
    44. 50. Ref:Harrison 17 e Cmdt 2011 eMedicine

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