henoch-schnolein purpura

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Your text here Your text here CASE PRESENTATION Your text here DR.KAILASH RAJ

Case History : 

Case History 50 yr old male Abdul Qadeer was admitted thru ER on 15feb 2011 with; He was in his usual state of health and used to sit and walk with help; that 2 days before getting admitted here he started vomiting; non projectile; contents were food particles & there was no blood. Besides that he had mild epigastric pain and one episode of hematuria. All of these were associated with loss of appetite, generalized weakness and body ache; so they rushed to ER and got admitted..

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Case History PM Hx: DM. HTN. ICH. BPH PS Hx: Not significant Personal Hx: Appetite---Decreased Bowel Habits---Regular Micturition ---- Hematuria Thirst---- Normal H/O Weight loss but not documented Family Hx: No history of T.B Parents: Deceased Siblings: Healthy 3 Children: Healthy Socio-economic Hx: Fair

Physical Examination : 

Physical Examination 50 year old male of average height & built, lying comfortably on bed; oriented to time place and person Pulse: 98/min B.P: 130/80 Resp: 16/min Temp: 100F Gen: Anemia+ve, Jaundice –ve, Dehydration –ve, Cyanosis –ve, Clubbing –ve, Oedema –ve. Neck: No JVD or hepatojugular reflux & no lymph nodes palpable Cardiovascular: S1 and S2 audible, no murmurs. Pulmonary: B/L NVB, No wheeze, Crepts Abdomen: Soft, non tender, no visceromegaly

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Physical Examination CNS: GCS 15/15 and neck stiffness-ve, other signs of meningeal irritation -ve Bulk --- B/L Normal Power ---2/5 Lt side & 5/5 Rt side Tone --- Dec Lt side Reflexs--- Diminished Lt side Plantars--- Rt ↓ ----- Lt- ↑ Dermatologically: When he got admitted here he was normal but on the very 6th day, he started having pinkish maculopapules specifically on upper and lower limbs and within 2 days it progressed and became purpura.

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Physical Examination (Skin Involvement)

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Laboratory reports

Laboratory reports : 

Laboratory reports

Treatment given : 

Treatment given Inj: Risek Inj: Gravinate Inj: Augmentin Inj: Amikin Syp: Duphalac Tb: Piozer Tb: Getryl Tb: Lipiget Tb: Oflobid (for 1day) Tb: Xatral LP Tb: Atarax Tb: Osran Nerisone cream

Differential Diagnosis : 

Differential Diagnosis Meningococcal meningitis or septicemia Idiopathic thrombocytopenic purpura Drug reactions Rocky Mountain spotted fever Familial Mediterranean fever Kawasaki disease Henoch –schonlein purpura

Urinary Tract Infection Henoch-Schonlein Purpura : 

Urinary Tract Infection Henoch-Schonlein Purpura

Henoch-Schonlein Purpura : 

Henoch-Schonlein Purpura

Henoch-Schönlein Purpura (HSP) : 

Henoch-Schönlein Purpura (HSP) Also known as; Anaphylactoid purpura Purpura rheumatica Schönlein–Henoch purpura It is a form of vasculitis; affects the small vessels called capillaries in the skin and frequently the kidneys.

Peeping Into Past : 

Peeping Into Past First description was by Heberden in 1806 Schonlein first described the association of arthralgias and purpura, and he termed it ‘peliosis rheumatica’ in 1837. Henoch (Schonlein’s student) described GI and renal manifestations In 1874 and 1899 respectively.

Epidemiology : 

Epidemiology More frequent in children than adults, with most cases occurring between 2 and 8 yr of age. Most frequently in the winter and spring months; 50% follow a URTI The overall incidence is estimated to be 9-18/100,000 population. Males are affected twice as frequently as females.

Etiology : 

Etiology Unknown! Possible offenders: URI: half of pediatric cases linked to streptococcal infection Malignancy: lymphoma, carcinoma Viral: hepatitis, parvo B19 Vaccines: hept B vaccine Meds: quinolones, clarithromycin

Precipitating factors that have been reported… : 

Precipitating factors that have been reported… Infections URI Measles Rubella Parvovirus B19 Mycoplasma Coxsackie virus Toxocara Amebiasis Salmonella C.difficile H.pylori Adenovirus Legionella Tuberculosis Mumps Streptococcus Morganella morganii Drugs Vancomycin Streptokinase Ranitidine Cefuroxime Diclofenac Enalapril Captopril Others Food hypersensitivity Cold exposure Autosomal recessive Chronic granulomatous disease Myelodysplastic syndrome Small cell lung cancer Breast cancer

Pathogenesis : 

Pathogenesis Its a small-vessel leukocytoclastic vasculitis characterised by the tissue deposition of IgA-containing immune complexes within affected organs. Symptoms of HSP are a consequence of the usual location of the acute small vessel damage primarily in the skin, gastrointestinal tract, and kidneys. Skin biopsies of lesions show neutrophils and monocytes as the predominant cell typesLikely 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

CLINICAL FEATURES : 

CLINICAL FEATURES The disease onset may be acute or insidious, with sequential occurrence of symptoms over a period of weeks or months Tetrad of symptoms Rash (Palpable purpura) (95-100%), especially involving the legs, may not be present on initial presentation Abdominal pain and vomiting (85%) Arthritis/arthralgias (60-80%), – more common in adults and most common in knees and ankles Renal disease (21-54%) Subcutaneous edema (20-50%) Scrotal edema (2-35%)

RASH : 

RASH Most commonly seen on lower extremities and buttocks, also seen on the trunk and arms. Beginning as erythematous pinkish maculopapules that initially blanch on pressure and progress to petechiae or purpura, Characterized clinically as palpable purpura that evolve from red to purple to rusty brown before they eventually fade Last from 3-10 days, and may appear at intervals that vary from a few days to as long as 3-4 mo. In <10% of children, recurrences of the rash may not end until as late as a year Damage to cutaneous vessels also results in local angioedema, which may precede the palpable purpura. Edema independent of purpura occurs primarily in dependent areas such as below the waist, over the buttocks (or on the back and posterior scalp in the infant), or in areas of greater tissue distensibility such as the eyelids, lips, scrotum, or dorsum of the hands and feet.

Few examples of Rash… : 

Few examples of Rash…

Few examples of Rash… : 

Few examples of Rash…

Gastro Intestinal INVOLVEMENT : 

Gastro Intestinal INVOLVEMENT Vasculitis anywhere in the GI tract More common in children. Symptoms include Colicky abdominal pain Nausea Vomiting Diarrhea Constipation or Bowel intussusception. May present with GI bleeding due to submucosal hemorrhage/edema Usually 1 week after appearance of rash Complications: gastrointestinal hemorrhage, bowel ischemia and necrosis, intussusception (children only), and bowel perforation 54% are hemoccult positive, but rare to have frank hemorrhage.

Arthralgia/Arthritis : 

Arthralgia/Arthritis Knees and ankles most common Transient, migratory, and oligoarticular No joint space destruction No longstanding sequlae

RENAL INVOLVEMENT : 

RENAL INVOLVEMENT In up to 50% of patients Usually more rapidly progressive in adults. Rare in children May present with hematuria or proteinuria Can have mild glomerulonephritis leading to microscopic hematuria and can lead to a rapidly progressive glomerulonephritis with RBC casts It may lead to chronic hypertension or end-stage kidney disease in the future Usually resolve spontaneously. Increased serum levels of insulin-like growth factor (IGF)-1 and IGF-binding protein-3. So these could be new markers for determination of renal involvement in HSP. Tohoku J Exp Med. 2008 Apr

Complications : 

Complications

DIAGNOSTIC EVALUATION : 

DIAGNOSTIC EVALUATION Generally a clinical diagnosis Routine laboratory tests are neither specific nor diagnostic CBC, Urine D/R, Creatinine Level, PT/APTT Anemia may result from chronic or acute gastrointestinal blood loss May have mild leukocytosis and thrombocytosis Raised ESR Normal serum complement levels Elevated IgA in 50% Ultrasound Abodmen 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

MANAGEMENT : 

MANAGEMENT Usually self-limiting (1-6 weeks) NSAIDS: increased risk of GI bleed Steroids: Prednisone 1 mg/kg Helps with arthralgias and abdominal sxs Not proven to benefit skin or renal disease Does not shorten duration of active disease Does not prevent recurrence Severe renal disease: ‘limited evidence’ Cyclophosphamide, Plasmapharesis IVIG Cyclosporin Symptomatic management of GI symptoms and surgical intervention if needed.

Indication to start systemic prednisone : 

Indication to start systemic prednisone 2% of the patients (0.1–0.4 per 100 000 per year) develop (haemorrhagic) bullous lesions. These can spread over the whole body and can result in ulcers, necrosis and scars.

Prognosis : 

Prognosis HSP is generally a benign disease with an excellent prognosis. More than 80% of patients have a single isolated episode lasting a few weeks. Approximately 10-20% of patients have recurrences. Fewer than 5% of patients develop chronic HSP. Abdominal pain resolves spontaneously within 72 hours in most patients

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The future belongs to those who believe in the beauty of their dreams.Eleanor Roosevelt THANKS

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