a case of henoch-schonlein purpura
TRANSCRIPT
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PROF.DR.NOORUL AMEENDR.R.PANDICHELVAN
M6
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23 yrs old male patient admitted on 5.2.11
in surgical ward with
H/o abdominal pain 14 days
more in right lower quadrant
colicky
intermittent pain no radiation
not related with food intake
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No h/o vomiting
No h/o fever
No h/o diarrhoea
No h/o jaundice
No h/o cough
No h/o dysuria
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On examination pt Conscious
Oriented
Afebrile Ao /Jo/Cy o/Cl o
CVS S1S2
RS NVBS
P/A tenderness in right iliac fossa
CNS NFND
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clinically diagnosed as Acute appendicitis and treated with
NPO IVF DNS and NS INJ Ciprofloxin 200mg iv bd INJ Metronidazole 500mg iv tds INJ Ranitidine 50 mg iv bd
Planned for surgery
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Patient developed skin rashes in both lower extremities and physician opinion was obtained for a/c appendicitis with ? drug allergy and fitness for surgery
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General examination:Pt Conscious Oriented Afebrile I * P * CY * CL * LNE * Purpura – both lower legs upto
thigh and in both upper limbs -palpable ;confluent
Bp 110/80 mmHg PR 86/min CVS S1S2 RS NVBS CNS NFND
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PICTURE
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P/A Not distended Moves with respiration Flanks free No distended veins No visible peristalis Soft Tender No organomegaly No free fluid BS heard
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Past history: Patient admitted in GRH for the c/o arthralgia and skin rashes 10 days ago
diagnosed as post viral arthralgia ; treated and discharged
Not k/c/o DM; HT; PTPersonal history: Not an alcoholic;smokerPatient took over to medical ward
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Provisional diagnosis
A/C abdominal pain with purpura for evaluation
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Pt managed with IVF DNS 2 pints Inj.Ciprofloxin 200 mg iv bd Inj.Metronidazole 500 mg iv tds Inj.Ranitidine 50 mg iv bd Tab.Paracetamol 500mg tds
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Differential diagnosis for medical causes of pain abdomen
Diabetic ketoacidosis Herpes zoster Sickle cell anemia A/c intermittent porphyria Poly arteritis nodosa Henoch schonelin purpura Lead poisoning Hyperlipidaemia Allergic pain [C1 esterase
inhibitor deficiency]
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Differential diagnosis for palpable purpura 1. Vasculitis a.Cutaneous small vessel
vasculitis B.Polyartritis nodosa C.Essential mixed
cryoglobulinemia 2.Emboli A.A/c menigococcemia B.Disseminated gonococcal
infection C.Rocky mountain spotted fever D.Ecthyma gangrenosum
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Urine Albumin- trace
Sugar - nil Deposit- 1-2pc/hpfCBC HB- 12.2GM/DL TC-6400 DC – P 65; L 32; E 3 ESR -8/12 PLC 345000 MCV 82.2 MCH 24.5 MCHC 31.2 PCV 38 %
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RFT : Urea -14 Creatine- 0.6 RBS – 87 Na -132 K-3.7 Cl -94 Bicarbonate-18
ECG : Normal
CXR : Normal
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USG abdomen : Normal except for probe tenderness ; no appendicular mass
CT abdomen : Peri appendicular edema seen suggestion: a/c appendicitis
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SP 3 09.02.11CBC Platelet count -424000 TC -12800 cells/ cumm RBC -5.51million/ul DC – P78; L12; E10 PCV -45.7% MCV -32.9 MCH - 26.9 MCHC -32.4 HB -14.8 ESR -30mm/hr
Prothrombin time 14 sec APTT 26 secINR 1.0
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BIOCHEMISTRY
Sugar -87 Urea – 21.8 Creatine – 0.74
LIVER FUNCTION TEST
Total bilirubin -0.52 Direct bilirubin -0.20 AST -25 u/l ALT -17u/l GGT – 17u/l SAP - 160u/l Total protein -7g/dl Albumin – 4g/dl Globulin -2.9 g/dl
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ELECTROLYTES: Na -141 K - 4.89 Cl -103.4SEROLOGY HBSAG – Negative ANTIHCV- Negative HIV - Negative URINE MICROSCOPY Leucocytes -3-4/hpf Ep cells - occassional RBc -2-3/hpf SERUM LDH 219 u/l
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Urine analysis 14.02.2011 Glu -trace Bil -neg Ketone neg Sg - 1.015 Blo 2+ ph 7.5 Pro 3+ Nit neg Leu neg STOOL Ova nil Cyst nil occult blood nil
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IMMUNOLOGY
ANA 1:10 NEG 1:40 NEG ASO NEG RF NEG CRP NEG C ANCA NEG P ANCA NEG Ig A 391mg/dl [ normal
80-350 mg/dl]
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Histopathology reportSpecimen:purpura right forearm biopsyMicroscopic description:stratified squamous
epithelium with hyperkeratosis , subepithelium shows congested blood vessels infiltrated with lymphocytes
Impression : cutaneous small vessel vasculitis
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DIAGNOSIS
HENOCH SCHONLEIN PURPURA
Patient treated with Tab. Prednisolone 1mg/kg Tab.Ranitidine 150 mg
bd Tab.BCT tds
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HSP is characterized by a classic tetrad of
Non thrombocytopenic palpable purpura
Arthralgia or arthritis
Gastrointestinal and renal involvement
Rarely involvement of lungs , cns,genitourinary tract
Cutaneous involvement is most common presentation
Also called anaphylactoid purpura
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EPIDEMIOLOGY90% of cases reported in children
Peak in children aged 4-7
Male:Female (1.5:1)
50% follow a URI
Renal disease is more severe in adults
Seasonal variation ; peak incidence in spring
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PATHOGENESISLikely mechanism thought to be an immune-
complex mediated disease with deposits in the glomerular capillaries, dermal capillaries and GI tract.
Mesangial deposits of IgA are the same as those seen in IgA nephropathy
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PRECIPITATING ANTIGENS
INFECTIONSURIMeaslesRubellaParvovirus B19MycoplasmaCoxsackie virusToxocaraAmebiasisSalmonella
C.difficileC.difficile
H.pyloriH.pylori
AdenovirusAdenovirus
LegionellaLegionella
TuberculosisTuberculosis
MumpsMumps
StreptococcusStreptococcus
Morganella Morganella morganiimorganii
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PRECIPITATING ANTIGENSDrugs
VancomycinStreptokinaseRanitidineCefuroximeDiclofenacEnalaprilCaptopril
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PRECIPITATING ANTIGENSOther:
Food hypersensitivityCold exposureAutosomal recessive Chronic granulomatous
diseaseMyelodysplastic syndromeSmall cell lung cancerBreast cancer
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CLINICAL FEATURESPALPABLE PURPURA: most commonly seen
on lower extremities and buttocks, however can also been seen on the trunk and arms. Lesions begin as erythematous macules and
progress to purpuric, non-blanching, nonpruritic lesions that may become confluent
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Gastrointestinal manifestationsOne third with HSP have GI featuresColicky pain, nausea, vomiting, GI bleed50% have occult bleedingMajor GI bleed in 5% Intussusception 2%Abdominal pain: sub-mucosal and intramural
extravasation of fluid and bloodEndoscopy: Erosive duodenitis
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Renal manifestationsAbnormalities of urinary sediment
Acute nephritis
Progressive renal disease Glomerular crescent formation - fatal outcome
Mortality 4%
Nephropathy adults 85%; children 25%
Renal insufficiency 13% in adults, 0% in children
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HSP in Children & AdultsFEATURES 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
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DIAGNOSTIC EVALUATIONMay have mild leukocytosis
Normal platelet count
Occasionally eosinophilia
Normal serum complement levels
Elevated IgA in 50% cases
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DIAGNOSISGenerally a clinical diagnosis
Skin Biopsy: can be helpful and used to confirm IgA and C3 deposits and leukocytoclastic vasculitis.
Renal Biopsy: not usually needed for diagnosis. Will show mesangial IgA deposits and segmental glomerulonephritis
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DIAGNOSTIC CRITERIA
ACR criteria-1990
three or more of the following cricteria 1.age 20 yrs or less at disease onset 2.palpable purpura 3.a/c abdominal pain with git bleeding 4.biopsy show granulocytes in the walls
of small arterioles and venules in superficial layers of skin
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EuLAR/PReS criteria -2006 mandatory criterion 1. palpable purpura plus at least one of the following criteria 1.diffuse abdominal pain 2.iga deposition in any biopsy 3.arthritis or arthralgia 4.renal involvement[hematuria and or
proteinuria]
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Chapell hill consensus group classification system
primarily use nonclinical criteria
require only presence of small vesel vasculitis with IGA deposition
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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
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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.
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MANAGEMENTUsually self-limiting (1-6 weeks)Steroids:
may decrease tissue edema, may aid in arthralgias and some abdominal pain
Has not been shown to be beneficial in kidney disease or dermal manifestations
Does not lessen chance of recurrenceDoes not shorten duration of disease
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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
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Ref:Harrison 17 e Cmdt 2011 eMedicine