CASE PRESENTATION

THE MAN WITH MALAR RASH

AND FACIAL PUFFINESS.
BY: E. A ADOMAKO

SUPERVISOR: DR. BEN EGHAN
12/15/2010
TGBTG
HISTORY
Forty-seven year old man, known alcoholic
with 7 pack years of smoking cigarettes
was well until 3 months ago when he felt he
easily became fatigued and breathless with
activities he could previously undertake
with ease. At the same time he experienced
occasional dizziness and regular
palpitations.
TGBTG
HISTORY
Within a week he noticed he had early
morning facial puffiness which resolved
during the day, as well as bilateral painless
pedal swelling, abdominal distention and a
sense of early satiety. He experienced
orthopnoea but had no paroxysmal
nocturnal dyspnoea.
TGBTG
HISTORY
. In addition, he had anorexia, metallic taste
with sialorrhea, painful swollen joints,
migratory in nature and bone pain
especially of both knees.
Initially he resorted to herbal medication
and drank herbal mixtures prepared from
mahogany( Meliacae family) for at least 2
weeks with no improvement.
TGBTG
HISTORY
He reported at KATH and was diagnosed
with hypertension and discharged with
antihypertensives(unspecified).
Four days later he was brought in with an
acute exacerbation of breathlessness with
cough productive of brownish sputum and
was subsequently admitted.
TGBTG
HISTORY
Systemic review revealed that he had
pruritus, nocturia, frothy urine, hesitancy.
He had no fever, seizures, jaundice and
haematuria.
Past medical history: He has no history of
diabetes mellitus, sickle cell
disease,pyelonephritis, admissions and
blood transfusions.
TGBTG
HISTORY
He has never had any radiologic procedure
with radiocontrast medium. No known
history of syphilis, hepatitis B and HIV. He
has a history of multiple bee stings. He has
had recurrent episodes of epistaxis since
childhood.

TGBTG
HISTORY
Drug history: He takes Ibuprofen frequently
for musculoskeletal pain. No history of
recent administration of procainamide,
hydralazine, quinidine and isoniazid.
Family history:
There is a family history of hypertension.
No known family history of diabetes or
kidney disease.


TGBTG
HISTORY
Social History:
He has been divorced for about a year. He
has six children and is a farmer. He stopped
smoking cigarette and drinking alcohol
about 4 months ago. He is registered with
the NHIS.

TGBTG
EXAMINATION
On examination was a middle aged man
who looked unwell and was lying propped
up in bed. He was conscious, alert and well
hydrated. He had a hypopigmented rash on
the malar eminences. He was pale, anicteric
with no palpable lymph nodes. He had
bilateral, pitting and non-tender pedal
oedema up to the knee. No clubbing,
Dupuytrens contracture, spider angioma,
palmar erythema or leuconychia.
TGBTG
EXAMINATION
CVS: Pulse was 80bpm, regular, of good
volume and non-collapsing. BP:
140/85mmHg
Apex beat was in the left 6
th
intercostals
space, a centimeter lateral to the mid-
clavicular line. He had no parasternal
heaves nor thrills. Heart sounds S1 and S2
were present and normal together with a
third heart sound. He did not have a
murmur.

TGBTG
EXAMINATION
Respiratory: He had no chest deformities,
chest expansion was equal. Tactile fremitus
was normal. Air entry was adequate, breath
sounds were bronchial with expiratory
wheezes bilaterally.



TGBTG
EXAMINATION
Abdomen: Full and moved with respiration.
No areas of tenderness on palpation. Liver
span 15cm, soft smooth and nontender.
Spleen and kidneys not palpable. Percussion
note was tympanic.
CNS: No defects in cognition, memory,
cranial nerves and motor system.

TGBTG
DIFFERENTIAL DIAGNOSES
: Chronic kidney disease secondary to:
Systemic lupus erythematosis( Malar rash,
migratory arthritis, renal disease, ANA?)
Wegeners granulomatosis
Good pastures disease
Rheumatoid arthritis

TGBTG
INVESTIGATIONS
Urinalysis
Blood Urea and
electrolytes
Liver function test
Anti-nuclear antibody
Anti-double stranded
antibody

Full blood count
ECG
Chest x-ray
ESR
CRP
TGBTG
CHEST X-RAY
TGBTG
CBC
TGBTG
OTHER LABS
PARAMETER RESULTS REFERENCE VALUE
CREATININE 1056 UMOL/L 61-155
UREA 33.59 UMOL/L 2.3-7.6
BUN:CREATININE
RATIO
0.032
ANTI-NUCLEAR
ANTIBODY
1:80(POSITIVE)
URINE PROTEIN ++++
TGBTG
TREATMENT
TB NIFECARD 30mg bd(14days)
LISINOPRIL 10mg dly(14days)
ALDOMET 250mg(14days)
PREDNISOLONE 60mg dly(14days)
HYDROXYCHLOROQUINE 500mg
dly(14days)
METHOTREXATE 7.5mg wkly(4wks)
IV FRUSEMIDE 80mg b.d(5days)

TGBTG
DISCUSSION POINTS
TGBTG
RENAL MANIFESTATIONS
OF SYSTEMIC DISEASE
The kidney is often involved in systemic
disease.
Renal dysfunction also presents with
systemic manifestations of disease.
Eg. Heart failure Acute Kidney Injury
Chronic Kidney Disease Pericarditis
CKD Hypertension
TGBTG
SLE
Renal component part of SLE even if not
clinically obvious.(lupus nephritis)
Immune complex deposition in GBM
activation of complement inflammatory cell
invasion.
Presents as proteinuria( might be in nephrotic
range), haematuria and glomerular casts.
TGBTG
References
Renal Manifestations of systemic disease(2009) PatientUK
retrieved 15
th
November,2010 from
http://www.patient.co.uk/doctor/Renal-Manifestations-of-
Systemic-Disease.htm
Longmore M., Wilkinson I., Turmezei T. Cheung
K.C,(2008) Oxford Handbook of Clinical Medicine,
Oxford UK. Oxford University Press.
KUMAR V, ABBAS K.A, FAUSTO N
(2005).PATHOLOGIC BASIS OF DISEASE. ELSEVIER
SAUNDERS, PHILADELPHIA
Boon A.N,Colledge R.N, Walker B.R(2005).DAVIDSONS
PRINCIPLES AND PRACTICE OF MEDICINE.
ELSEVIER SAUNDERS, PHILADELPHIA



TGBTG
Closing thought..
The aim of medicine is to prevent disease and
prolong life, the ideal of medicine is to
eliminate the need of a physician.
William J. Mayo (1861 - 1939)

THANK YOU
TGBTG
Thank you

TGBTG
DIABETIC NEPHROPATHY
The most common systemic manifestation of systemic disease
is diabetic nephropathy.
Commonly presents after about 10yrs of DM.
Starts as microalbuminuria (30-300mg/day)
Progresses to frank albuminuria(>300mg/day)
Hyperglycaemia alters cytokine activity.
Initially,hyperfiltration,mesangial expansion, Bowman C
thickening, glomerular hypertrophy.: RAAS, endothelin
activation, PKC, TGF-B and abnormal polyol metabolism.
Rx: Glucose control, control BP and use ACEi even in
normotensives.
TGBTG
HYPERTENSIVE NEPHROPATHY
of patients presenting with ESRD
Nephrosclerosis occurs as a result of ischaemia.
Accelerated by malignant hypertension.
Hyperfiltration results in intraglomerular
hypertension.
Patient hypertensive for about 10yrs.
Patients present with other complications(eg. HF,
retinopathy, MI)
Treatment is with ACEi +other antihypertensives.
But if RAS, then ACEi is contraindicated.

TGBTG
RENAL DYSFUNCTION ASSOCIATED WITH
VASCULITIDES
Small and medium sized Vasculitides often
associated with ANCA.
ANCA activates neutrophils, which release
mediators with damage to blood vessels.
Usually cause RPGN. GFR falls by 50% within
2-3mths.
Clinically presents as flu-like symptoms,
migratory arthritis, myalgia, fever, anorexia,
weight loss, abdominal pain and skin nodules.
TGBTG
VASCULITIDES
PAN: Necrotizing inflammation typically
involving renal arteries but sparing pulmonary
vessels.
Microscopic polyangiitis is similar to PAN but
has pulmonary involvement.
Wegeners granulomatosis: Classicallyinvolves
the nose,sinuses, lungs,and kidneys.(C-ANCA
positive. Saddle nose deformity.
Churg-Strauss: Allergic asthma , eosinophilia
with renal impairment.
TGBTG
Progressive Systemic Sclerosis
Causes renal impairment via microangiopathy
or renal crisis.
Renal crisis presents in patients with sudden
onset of diffuse dermatological involvement.
Presents as oliguria, hypertension, headache,
rapidly rising creatinine and peripheral oedema.
Usually occurs within the first 4yrs of diagnosis.
Watch out for it and treat with ACEi.
TGBTG
MULTIPLE MYELOMA
Monoclonal AB from transformed plasma
cells.
Damage from: light chain deposition, amyloid
deposition, hypercalcemia, TLS, cast
nephropathy and dehydration.
It might be the presenting feature of MM.

TGBTG
HUS
Most common cause of AKI in children.
85% make full recovery, 15% progress to
CKD.
Triad of Haemolytic anaemia,
thrombocytopenia and renal failure.
Infection with E. coli, Shigella, Salmonella,
Yersinia, Campylobacter and URTI.
GI bleeding and petechiae.
Rx is supportive+renal replacement therapy.
In severe cases: plasma exchange may be
considered.
TGBTG
SICKLE CELL DISEASE
Postmoterm series in adults with SCD found that 20% died of
renal failure.
The disease causes glomerulopathy with proteinuria, progressive
renal insufficiency and ESRF. Papillary necrosis is another
mechanism of injury.
Children present with hyposthenuria, polyuria and nocturia.
Incipient albuminuria is a good marker of disease.
Management is by managing SCD to prevent renal
complications
TGBTG
Good pastures Disease
Acute glomerulonephritis with pulmonary
component( haemoptysis/ pulmonary
haemorrhage)
Anti-GBM
Treatment is supportive, immunosuppressives
and plasmapheresis.
TGBTG
OTHERS
Cryoglobinuria
Sjgren's syndrome
HIV
Hepatitis B, syphillis
Several other diseases including
haematologic, oncologic,immunologic and
metabolic diseases have renal
manifestations.
TGBTG