MONTHLY RHEUMATOLOGY

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MONTHLY RHEUMATOLOGY MEETING CASE
PRESENTATION DR BERYL DSOUZA HOSPITAL
SEREMBAN
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History
PVR,17 yr old Indian male referral from UH, as
Stills Disease. Presenting complaints 1.Wt loss
approx 20 kgs over 3 mths with fever 2.Arthritis
of 2nd PIPDIP jts,1st MCP jt 3rd DIP jt
for 2 mths preceded with 1 wk of AGE 3.Rash
palm for 2 mths
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Past History 1.Fever in Aug 2003 for 2wks with
diarrhoae. 2.Ulcers both ankles at year
2000 Family HistoryNil sig. Social History Form
4 student Youngest
child Non smoker/non
alcoholic Not sexually
active
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Examination Emaciated ,wt 44 kgs Afebrile BP
120/90mmHg PR 100/min Pale, no
jaundice. Fundoscopy normal Heart/Lungs/Per
Abdomen-Normal Musculoskeletal System 1.Swelling
with hyperpigmentation ankle 2.Tender 2nd
PIPDIP jts 3.Tender shoulder.
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Problems 1.Rapid wt loss with fever 2.Anaemia 3.A
rthritis of hand jts 4.P/H of ulcer both ankles
? Vasculitic Ulcer
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• Provisional DiagnosisJuvenile Idiopathic
  Arthritis.
• Diff Diagnosis Hematological Malignancy
• Arteritis / Vasculitis

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Investigations

• X-rays shoulder-normal.
• Handsperiarticular
• osteopenia,reduced jt space at
  2nd PIP,3rd PIP jts with erosions
• CXRnormal.
• Bld c/s-no growth.
• Urine FEME-no proteinuria/haematuria.
• Urine c/s-no growth.

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Investigations

HbsAg-non reactive,anti HCV-non reactive,IgG
HAV- Non reactive. Stool ova/cyst-neg,stool
c/s-no growth. Rh factor-neg. ANA-neg, ANCA-
neg S.C3/C4-1.69/0.41 FBP-Iron def anaemia, Hb
10 CRP 61.3, ESR 73 S.Fe-17.81,TIBC-58.01 OGDS-Hia
tus hernia. Colonoscopy-normal Us
abd-hepatomegaly,bilat renal parenchymal
disease. 2D-Echo Heart-Normal
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Progress (R) arm (L)arm (R)arm
(L)leg BP rising 170/110 160/110
155/98 160/90 Wt loss of 2kgs,spike of
temp of 37.9c Normal Urine FEME Polyarthritis
Nodosa suspected. RenalMesenteric angiogram
done in HKL on 28/4 showed microaneurysms
consistent with P.A.N
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Final Diagnosis Polyarterits nodosa Criteria 1.W
t lossgt4 kgs 2.New onset diastolic
BPgt90mmHg 3.Characteristic arteriographic
abnormalities not resulting from non
inflammatory processes
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Treatment

• Oral Cyclophosphamide 2mg/kg
• T.Prednisolone
• Anti hypertensives
• T.Bactrim

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Progress

• Initially Good Response
• Suffered epileptic seizures-1st on 4/6
• CT Brain-(Lt) Pontine Infarct.
• Started on C Phenytoin-100mgs tds, and
  Carbamazepine,T.Cyclophosphamide, seizures not
  well controlled
• Changed to I/V Pulse Cyclophosphamide 750mg/M²
  BSA

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Investigations 2/4 9/4 19/5 22/6
13/7 25/8 9/9 24/11 7/12 9/3/05 TWBC9.1
12.8 10.8 7.7 4.4 6.5 4.9 7.0
3.0 4.1 HB 10.8 11.6 11.2 13.2 13.7 12.4
10 11 15.5 15.0 PLT 372 354 320
287 329 264 312 195 271 218 CRP
61.3 51.4 28.8 50.4 15.3
1.4 4.0 ESR 73 56
20 50 11 13
12 ALT 66 86 20 32
55 AST 44
41 41 71
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RENAL PROFILE-NORMAL




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Well since then with wt gain of 30 kgs and BP
controlled. Current medicationT.Metoprolol-5
0mgsbd
C.Phenytoin-300mgson
T.Carbamazepine-200mgsbd
T.Prednisolone-5mgs od
T.FA-5mgsod
T.VitC-100mgsod

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Polyarteritus nodosa REFUpToDate Introduction Po
lyarteritis nodosa is a systemic necrotising
vasculitis typically affecting the small and
medium sized arteries In some cases,only smaller
vessels are affected in the condition called
microscopic polyarterits or microsco pic angitis
associated with presence of ANCA.
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Clinical Manifestations 1.Neurologic
diseaseMononeuritis multiplex,ischemic stroke,
or intracerebral hemorrhage. 2.Skin
diseaseLivido reticularis,skin
ulcers,bulious/vesicular eruptions.

3.Renal diseaseRenal infarctions,Hypertension
secon dary to renal ischemia. 4.Gastrointestinal
disease Mesenteric arteritis,bowel in farction
with perforation,nausea,vomiting,malena, Bloody
or non bloody diarrhoea. 5.Coronary artery
diseaseMyocardial ischemia, Congestive heart
failure.
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6.Muscle diseaseMyalgia,muscle
weakness,s.creatinine kinase may be
elevated. OthersOrchitis,toxic retinopathy with
hemorrhages, exudates and retinal
detachment. Breast and uterine involvement. Pulmon
ary involvement. Etiology and Diagnosis Most
cases idiopathic. Hepatitis B infection and hairy
cell leukemia may be pathogenetically important.
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Classification criteria
10 criteria established by ACR. Sensitivity and
specificity for diagnosis of poly arteritis of
82 and 87 found in a patient with documented
vasculitis with at least 3 of below
1.Otherwise unexplained wt lossgt4kg 2.Livido
reticularis. 3.Testicular pain or
tenderness. 4.Myalgias.weakness of
muscles,tenderness of leg muscles or
polyneuropathy. 5.Mononeuropathy or
polyneuropathy. 6.New onset diastolic blood
pressuregt90 mmHg 7.Elevated levels of s blood
urea nitrogengt40mg/dl or 14.3 mmol/l or
creatinine.gt1.5mg/dl or132mimol/l
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8. Evidence of Hepatitis B virus
infection. 9.Characteristic arteriographic
abnormalities not resulting from noninflammatory
disease processes. 10.A biopsy of small or
medium-sized artery containing polymorphonuclear
cells. Tissue biopsy Diagnosis must be
confirmed by biopsy of a clinically affected
organ such as peripheral nerve,testis,skin or
kidney. Renal biopsy in classic polyarteritis may
reveal pathognomonic inflammation of medium sized
arteries.
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Treatment Prognosis in untreated PAN is poor
with 1 yr and 5 yr survival rates approx 50 and
13. Renal failure,mesenteric,cardiac or cerebral
infarction are major causes of death. The
following recommendations apply to patients with
idiopathic disease. Initial therapy Corticostero
id therapy beneficial in some patients.
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Response seen within first 3 months. Longterm
remissions can be induced in most patients wth
cyclophosphamide. Combination of corticosteroid
and cyclophosphamide improves survival in
patients with more severe disease when compared
to steroid theraphy alone. Patients with mild and
stable large vessel disease treated initially
with prednisolone at dose of 1mg/kg per
day. Those who do not respond,develop side
effects or who
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initially have more serious disease should be
treated with cyclophosphamide and prednisolone in
a regimen similar to that used in Wegeners
granulomatosis. Duration of therapy Optimal
duration unknown. Usually treat for 1
yr. Hypertension Elevation of blood pressure
mediated by ischemia induced activation of renin
angiotensin system. ACEI may worsen renal
function due to removal of effect of angiotensin
2 on autoregulation. Prognosis Untreated
polyarteritis associated with poor prognosis- 13
5 yr survival rate.
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Outcome improved with therapy to approx 80
survival at 5 yrs. Most deaths due to active
disease within first 18 months of disease
onset. Renal insufficiency and gastrointestinal
disease most significant adverse prognostic
indicators. Renal transplantation Few data on
outcome of patients with PAN and ESRD who undergo
renal transplantation. In the ERA-EDTA
registry,transplantation was associated with
lower patient and first cadaveric allograft
survival rates compared with those with standard
renal disease.
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