Case PresentationCase Presentation

New Mansoura General Hospital (internationalinternational)-(Egypt(

Nabeeh Dr. Neveen

Nephrology Specialist Nephrology Specialist

35 year old female , housewife, married 8 years ago , has one daughter aged 6 years old. Has hx of abortion once at 10 weeks 7 years ago .

No special habits of medical importance

Family history: irrelevant

complaintcomplaint

The patient complaining of painful

rash distributed at trunk , upper and

lower limbs for one month duration.

Present historyPresent history

The condition started one month ago by gradual onset and progressive course of painful purpuric eruption with raised edge distributed at both upper, lower limbs and trunk associated with fever , decrease urine output , dysuria , myalgia and weakness.

Present history-ContPresent history-Cont

The condition also associated with

productive cough with sputum

whitish in color , exacerbating at

night and associated with dyspnea.

Past Medical HistoryPast Medical History

Hypertensive 10 years ago.

HCV +ve 5 years ago .

Stone former with history of stone removal from right kidney one year ago.

Recurrent documented UTI

ExaminationExaminationGeneral examination: the patient is alert

conscious oriented to time place and person of intact memory.

• No puffiness of the eye lid ,or yellowish discoloration of the sclera ,but there is painful ulceration of the tongue , whitish in color.

• Blood pressure: 160\90

• Pulse : 90 beat\ minute regular , equal in both sides , average volume

• BW 116.5 kg. BMI : 37

Examination contExamination cont--

There is painful purpuric papule with a raised edge distributed at the trunk and upper and lower limbs.

There is ulceration at the back of the leg.

Examination contExamination cont--

also there is bluish discoloration of little and 3rd toes of the right lower limb and 3rd toe of left lower limb associated with sever pain ,

There is mild pitting edema of both lower limbs extending to the knee, dorsalis pedis artery are not felt on both side

Examination contExamination cont--

Abdominal exAbdominal ex:• By inspection: raised purpuric eruption on the

skin of the abdomen. • By palpation : splenomegaly

CardiacCardiac exex: S1 + S2 + 0

ChestChest exex: there is decrease air entry with stony dullness in both sides of the chest .

Drug HistoryDrug History

Captopril 25 mg twice daily.Captopril 25 mg twice daily.

Amlodipin 5 mg once daily.Amlodipin 5 mg once daily.

Furosemide 40 mg once dailyFurosemide 40 mg once daily

InvestigationsInvestigations

CBCCBC• WBC: 11,000 • RBCS: 2.87• Hb : 6.9• HCT: 20.8• MCV:72.8• MCH:24.0• MCHC:33.1• PLT :99,000

S.Cr 6.3 mg/dl with a basal serum creat. 0.7 mg/dl

e GFR 10.3ml\min

Blood urea: 280 mg\ dl

S.Na 135 mg\ dl

S. K 3.9 mg\ dl

U.A 18.0 mg\ dl

I.N.R 1.55

ALT 10 mg\ dl

AST 19 mg\ dl

S.Albumin 3.2 mg\ dl

S.Ca 9 mg\dl

S.Po4 6.9 mg\dl

ANA -Ve

ANCA -Ve

complement level decrease C4 , normal level of C3

serum Cryoglobulin -ve

Rhumatoid factor +ve

Urine analysis

Albumin +++

Leucocyte estrase: present+++

WBC\ HPF <100

RBCS\ HPF 8-10

Casts: absent

urine culture and sensitivity urine culture and sensitivity :

E.Coli .

sensitive to imipenem

24 hour urine collection :

Volume 800 cc

Total protein : 3654 mg

Abdominal U\SAbdominal U\S::Average size liver with coarse echopattren . It shows

periportal fibrosis , normal patent PV, no definite focal lesion .

Prominent wall of the gall bladder with single stone about 8mm , no mud, normal CBD

Moderately enlarged spleen with uniform echo pattern, , no focal lesion

Normal size , shape of the both kidney. Normal cortical thickness.

Rt kidney shows mild back pressure and a lower calyceal stone about 12.5 mm

No ascites could be detected

Right and left lower limbs color Duplex Right and left lower limbs color Duplex was done and revealed :was done and revealed :

Weak flow is seen in the right lower part of anterior tibial artery

Patent right external iliac, common femoral , superficial femoral, popliteal, peroneal and post. Tibial arteries , they have thin walled atherosclerotic changes. They filled with color signal on color Duplex examination . They show triphasic flow pattern with color Duplex examination

No aneurysm or arteriovenous malformation could be detected

Normal left lower limb arterial system

Provisional DiagnosisProvisional Diagnosis : :

VasculitisVasculitis with AKI with AKI

Renal biopsyRenal biopsy

Was done in our hospital but its insufficient and difficult due to obesity of the patient,

Patient referred to another nephrology center for renal biopsy but its also was insufficient

SOSO

Skin biopsy was done and revealed:

Consistent with leukocytoclastic vasculitis associated with cryoglobulinemia

So the diagnosis is

Mixed Cryoglobulinemia

ManagementManagementThe patient receive 2 haemodialysis sessions due to

uremic symptoms

4 plasmapheresis sessions

Pulse steroid (Methylprednisolone) o.5 g daily for 3 days followed by oral prednisolone 60 mg daily

Cyclophosphamide 2mg\kg \day

Imipenem 0.5 g\ 12 h

Amlodipin 10 mg once daily

Skin BiopsySkin Biopsy

One of the least invasive ways of making the diagnosis of vasculitis .A minor procedure performed under

local anesthesia. The wound is closed with 1–2 stitches that are removed 7–10 days later.

.

Skin Biopsy: Timing, Technique, Skin Biopsy: Timing, Technique, and Choice of Lesionsand Choice of Lesions

Biopsy extending to the subcutaneous taken from the most tender, reddish, or purpuric

lesion. skin is the key to obtaining a significant diagnostic result and serial

sections are often required to identify the main vasculitic lesions.

The optimal time for a skin biopsy is < 48 hours after the appearance of a vasculitic lesion (If the biopsy is poorly timed, the pathologic features of vasculitis may be

absent)

KDIGOKDIGO Recommendations RecommendationsFor eGFR >50 mL/min/1.73 m2, pegylated interferon

and ribavirin

For eGFR 15 to <50 mL/min per 1.73 m2, monotherapy with pegylated interferon

For eGFR <15 mL/min per 1.73 m2 (including patients on hemodialysis); monotherapy with standard interferon 3million unite ,3 times per week, for 12 months has been used with a succes , that is dose adjusted for a glomerular filtration rate less than 15 mL/min per 1.73 m2.

Antiviral treatment is recommended for at least 12 months

AdditionalAdditional KDIGO KDIGO RecommendationsRecommendations

Among MPGN patients with nephrotic-range proteinuria and/or rapid loss of kidney function and an acute flare of cryoglobulinemia, one of the following therapies should be considered:

Plasma exchange (3 liters of plasma thrice weekly for two to three weeks)

Rituximab (375 mg/m2 per week for four weeks); OR

Cyclophosphamide (2 mg/kg per day for two to four months)

Plus Methylprednisolone pulses (0.5 to 1 g/day for three days). Treated with ACEI or ARB to reduce proteinuria and achieve target

blood pressure goals Relapses of systemic cryoglobulinemia and membranoproliferative

glomerulonephropathy may be treated with additional doses of rituximab.

Saadoun. Rheumatology 2007;46:1234–1242Saadoun. Rheumatology 2007;46:1234–1242