DR. CHAITANYA H. BALAKRISHNAN
GUIDES: DR. H.V. NATARAJU, PROFESSOR AND H.O.D.,
DR. YOGITHA C.
DR. PRADEEP C.
DEPT. OF GENERAL MEDICINE,
KIMS HOSPITAL
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A 27 year old male, Mr. J, came with the following:
CHIEF COMPLAINTS:
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Fever Since 10 Days
Joint swelling Since 7 Days
Rashes Since 6 Days
Facial Puffiness Since 2 Days
HISTORY OF PRESENTING ILLNESS:
 Fever- intermittent type, associated with chills
 Joint swelling- large and small joints, associated with severe joint
pain, progressive
 Rashes- started over the hands and feet, then spread to involve the
arms, legs, chest, back and abdomen, reddish lesions, not associated
with itching
 Facial puffiness- more in the morning, decreases as the day passes,
not a/w decreased urine output
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No h/o bleeding manifestations, cough,
breathlessness, throat pain
No h/o chest pain, abdominal pain, urinary/
bowel disturbances
No h/o headache, vomiting, giddiness
PAST HISTORY: no h/o similar complaints in the
past, no other co-morbidities
FAMILY HISTORY: nothing significant
PERSONAL HISTORY: nothing significant
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PULSE: 126/min
BLOOD PRESSURE: 140/86mm hg
RESPIRATORY RATE: 22/min
Edema present- bilateral lower limb edema upto
ankles, pitting
Facial puffiness present
No pallor, icterus, clubbing, lymphadenopathy,
cyanosis.
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SKIN AND MUCOSA:
 maculopapular rash present over upper limbs, chest,
back and abdomen –blanchable
 palpable purpura present over bilateral lower limbs
 petechiae over the soft palate
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JOINTS: swelling of ankle joints bilaterally,
proximal and distal interphalangeal joints of both
hands
PURPURA OVER WRIST, FOREARM
AND PALMS
PETECHIAE OVER BACK
PALPABLE PURPURA OVER LOWER LIMBS INCLUDING DORSUM OF FEET
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RESPIRATORY SYSTEM:
 No abnormality detected
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CARDIOVASCULAR SYSTEM:
 Tachycardia present, no murmurs
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ABDOMEN EXAMINATION:
 Hepatomegaly present 2cm below the R.C.M.( soft,
rounded borders, non tender), bowel sounds heard
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CENTRAL NERVOUS SYSTEM:
 No abnormality detected
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VIRAL EXANTHEMATOUS FEVER WITH
ARTHRITIS- ? ARBOVIRAL DISEASE
RICKETSIAL FEVER
VASCULITIS
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DAY 1:
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Hb: 14.7g%
TC: 21790 cells/mm3
DC: P82.9, L12, M4.3
ESR: 36mm/hr
Platelet count: 1.37 lakh/mm3
B. Urea: 28mg/dl
S. Creatinine: 1.3 mg/dl
Urine routine: alb++, sug nil, PC 15-20cells/hpf, RBCplenty
 ECG: sinus tachycardia
 CXR: normal
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DAY 1:
 QBC FOR MP: Positive for plasmodium vivax
 Peripheral smear: normocytic normochromic blood
picture with neurophilic leukocytosis and no evidence
of malarial parasites
 Sepsis work up: positive( CRP, S. Procalcitonin, FDP)
 Widal test,HIV, HbsAg, VDRL, Weil Felix test: Negative
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DAY 1:
 USG Abdomen and pelvis: Borderline splenomegaly,
Grade 1 Medical Renal Disease
 Blood C/S- no growth, Urine C/S- Enterococcus
 PT with INR- normal
 LFT:
TB
DB
OT
PT
ALB
GLO
ALKP
0.5
0.2
76
105
2.9
6.7
164
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SEPSIS- ? UROSEPSIS
COMPLICATED PLASMODIUM VIVAX
INFECTION
RICKETSIAL FEVER
VASCULITIS
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Patient was started on broad spectrum
antibiotics( piperacillin-tazobactum), artesunate
and primaquine, antipyretics, doxycycline, antiinflammatory drugs and IV fluids.
By day 3, patient was symptomatically better:
 reduction in the joint pains and swelling
 blanchable rash over the trunk and chest was fading
 Purpura remained persistent over both lower limbs
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DAY 3:
 TC: 15710/mm3
 DC: N79, L11, M5, E3
 Platelet count: 1.48 lakh/mm3
 S. Creatinine 1.2mg/dl
 S. Calcium 9.2 mg/dl
 S. Phophate 2.8mg/dl
 S. Albumin 2.2mg/dl
 Urine routine: alb ++, PC 13-15/hpf, RBC 25-30/hpf
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By day 5, patient was afebrile with complete
reduction in the joint swelling and disappearance
of the chest and abdominal rash but purpura
remained persistent.
Patient was continued on IV antibiotics as per the
urine C/S report.
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DAY 5:
 TC: 9900/mm3
 Platelet: 1.61 lakh/mm3
 S. Creatinine: 1.0 mg/dl
 Urine spot protein-creatinine ratio: 1.45(normal <1)
 24 hour urinary protein: 1750mg/dl(nephritic range)
 Skin biopsy of palpable purpura: leukocytoclastic
vasculitis
NEUTROPHILIC
INFILTRATES WITH
FIBRIN DEPOSITS
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? HENOCH SCHONLEIN PURPURA SECONDARY
TO ENTEROOCCUS INFECTION CO-INFECTED
WITH PLASMODIUM VIVAX MALARIA
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Patient was discharged after 1 week of hospital
stay and was to review with repeat urine routine,
24 hour urinary protein after 1 week.
After 1 week, urine routine showed persistent
albuminuria and hematuria with a 24 hour
urinary protein of 2265mg/dl.
Hence the patient was re-admitted for a renal
biopsy.
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Renal biopsy:
 Glomeruli- Segmental mesangial cell proliferation
with endocapillary proliferation with 25% crescent
formation
 Tubules- RBC’s in the lumen but no atrophy
 Vessels- mild hyperplasia with intimal hyalinosis
 Immunofluroscence- diffuse global fine granular
deposits of IgA and C3, few IgG and IgM
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FINAL DIAGNOSIS: Henoch Schonlein Purpura
with ISRDC classification IIIa without chronicity
AFFECTED
GLOMERULUS
MESANGIAL PROLIFERATION
PAS NEGATIVE
NORMAL TUBULES
WITH MESANGIAL
PROLIFERATION
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International Study Group of Kidney Disease in
Childhood, uses crescents as a critical factor. In
this schema, grade I has minimal alterations,
grade II is pure mesangial proliferation, and
grades III to V have focal segmental or diffuse
proliferation with <50%, 50 to 75%, or >75%
crescent formation, respectively. Grade VI has
glomeruli with a membranoproliferative pattern
of injury, found in 2% of biopsies.
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The patient was started on steroid pulse therapy
for 5 days(IV Methylprednisolone 500mg OD) and
discharged on oral prednisone for 6 weeks.
Patient is a regular at the follow up OPD and
latest urine routine revealing traces of albumin
with RBC’s 3-5.
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HENOCH SCHONLEIN PURPURA SECONDARY
TO ENTEROCOCCUS INFECTION WITH
PLASMODIUM VIVAX CO-INFECTION
Henoch-Schönlein purpura (HSP; also referred to as Schönlein-Henoch
purpura, anaphylactoid purpura, or purpura rheumatica) is an acute
immunoglobulin A (IgA)–mediated disorder characterized by a
generalized vasculitis involving the small vessels of the skin, the
gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs
and the central nervous system (CNS).
 It is a subset of necrotizing vasculitis characterized by fibrinoid
destruction of blood vessels and leukocytoclasis.
 The prevalence of HSP peaks in children aged 3-10 years, but the
condition is also seen in adults.
 The dominant clinical features of HSP include:
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cutaneous purpura
Arthritis
abdominal pain, GI bleeding, orchitis
nephritis.
In one half to two thirds of children, an upper respiratory tract infection
(URTI) precedes the clinical onset of HSP by 1-3 weeks.
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The most serious complication of HSP is renal
involvement, which occurs in 50% of older children
but is serious in only approximately 10% of patients.
In 80% of patients, renal involvement becomes
apparent within the first 4 weeks of illness. Overall,
2-5% of patients progress to end-stage renal failure
(ESRD).
The renal biopsy features include acute glomerular
lesions like mesangial hypercellularity, endocapillary
proliferation, necrosis, cellular crescents, and
leukocyte infiltration.
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Allergens, such as foods, horse serum, insect bites,
exposure to cold, and drugs (eg: ampicillin,
erythromycin, penicillin, quinidine, and quinine),
may precipitate the illness.
Infectious causes include:
 Bacteria (eg, Group A streptococcus, Haemophilus
parainfluenzae, Campylobacter Mycoplasma, Legionella,
Yersinia, Shigella, or Salmonella)
 Viruses (eg, adenoviruses,Epstein-Barr virus [EBV],
parvoviruses, or varicella-zoster virus [VZV]).
 Vaccines such as those against cholera, measles,
paratyphoid A and B, typhoid, and yellow fever have also
been implicated.
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TREATMENT:
 Management of HSP includes adequate hydration;
immediate discontinuance of any exposure to
antigenic stimulants and follow-up each week for the
first month, every other week for the second month,
and monthly thereafter until abnormal urinary
findings subside.
 Nephropathy is treated supportively. Patients’ fluid
and electrolyte balance should be monitored, their
salt intake should be restricted, and antihypertensives
should be prescribed when needed.
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TREATMENT:
 Methyl Prednisolone 1g/ day for 3 days(pulse therapy) followed
by prednisone in a dosage of 1 mg/kg/day for 2-4 weeks and
then tapered over 2 more weeks is used in HSP nephropathy.
 Other treatment regimens have included IV or oral steroids
with or without any of the following:
▪ Azathioprine
▪ Cyclophosphamide
▪ Cyclosporine
▪ Dipyridamole
▪ High-dose IV immunoglobulin G (IVIg)
▪ Danazol
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HSP is a disease that may affect the adult
population precipitated by viral/bacterial
infections or even allergens and drugs presenting
with rash, fever, abdominal and renal
involvement. The disease is self limiting and the
treatment is conservative with steroids or
plasmapheresis only in rapidly progressing renal
disease.
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“Henoch-Schönlein Purpura Nephritis”; Anup Rai,
Cynthia Nast And Sharon Adler; JASN 1999
“Henoch-Schönlein Purpura Nephritis”:
Pathophysiology, Treatment, and Future
Strategy; Jean-Claude Davin; CJASN 2001.
“Henoch-SchöNlein Purpura in Adults:; María
José López Meiller, Javier Alberto
Cavallasca, María del Rosario Maliandi,
and Gustavo Guillermo Nasswetter- PubMed
Clinics 2007.