Slide 1: 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..
Slide 3: 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
Slide 5: 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.
Slide 6: Physical Examination (Skin Involvement)
Slide 7: 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
Slide 31: The future belongs to those who believe in the beauty of their dreams.Eleanor Roosevelt THANKS