Alexandra David,MD

Spitalul Clinic Sfanta Maria –

UMF Carol Davila Bucuresti, Romania

BF, male, 29 years

Photosensitivity and Raynaud phenomena – 6 months

No other significant personal and familial history

Smoking status: never

-acute onset bilateral ankle swelling (2 weeks)

-digital necrosis left leg

Case report….

•84 bpm; 120/70 mmHg

•36.6 C

•Normal chest exam

•Pedal pulse present

VASCULITIS

Primary vasculitis – Panarteritis nodosa

Vasculitis associated with possible etiology

Secondary vasculitis - SLE

ANTIPHOSPHOLIPID SYNDROME

INFECTIOUS ENDOCARDITIS

THROMBOANGIITIS OBLITERANS

Other…cholesterol crystal embolism

Patient refuses skin biopsy!

Digital necrosis…possibile etiology

Case report – Lab results

WBC 5000/mm3, normal Ly

Platlets 154000/mm3

ESR 82 mm/h, CRP 67.8 mg/l ?

Procalcitonine, hemocultures -

Low C3, C4

pANCA, cANCA –

RF, Anti CCP Ab-

HVB, HVC, HIV screening: negative

Cryoglobulines –

ANA +++, Anti dsDNA +++ , Anti Sm +++ (high values)

Lupus anticoagulant, anticardiolipin Ab ++

Anti U1 RNP, Anti Ro, La Ab –

Chest X Ray, heart US: normal

Urine : NORMAL, negative proteinuria

Tumoral markers -

SLICC Classification Criteria

Clinical Criteria

1.Acute Cutaneous Lupus

2.Chronic Cutaneous Lupus

3.Oral or Nasal ulcers

4.Non-scarring alopecia

5.Synovitis

6.Serositis

7.Renal

7. Neurologic

8.Hemolytic anemia

9. Leukopenia (<4000/mm3)

10.Thrombocytopenia (<100.000/mm3)

Immunologic Criteria

1. ANA

2. Anti –ds DNA

3. Anti –Sm

4. Antiphospholipid Ab

5. Low complement

6. Direct Coomb’s test

• ≥ 4 criteria (≥1Clinical + ≥1 immunologic) OR

• Biopsy confirmed nephritis + ANA/a dsDNA

Petri M et al, Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria

for systemic lupus erythematosus. Arthritis Rheum. 2012 Aug;64(8):2677-86. doi: 10.1002/art.34473.

Diagnosis

Systemic Lupus Erythematosus

Secondary Antiphosfolipid Syndrome

Same unclarities…

Positive CRP?

Unusual clinical manifestations

- bilateral ankle swelling

- peripheral ulcers in SLE

Treatment:

Immunosuppressants

Solumedrol 500 mg iv

Cyclophosfamide 1 g iv

Vasodilator

Alprostadil (PGE1) 40 microg iv BID (4 weeks)

Anticoagulants

Clexane 0.8 IU BID

Acenocumarolum , INR 2-3

Other:

Omeprasole, MESNA, K suppliments

Co-trimoxazole

Evolution after 4 iv Cyclophosphamide

First presentation

2 months after…

4 months after….ESR 15 mm/h,

CRP 2 mg/l

Normal C3, C4

Normal CBC

ANA +

1 week after 4th course of iv Cyclophosphamide ….

Palpable purpura Acute onset knee effusion

ESR 120 mm/h, CRP 274 mg/l

WBC 15600/mm3

Platelets: normal

C3, C4 slightly lower

Normal urinary sediment

Negative proteinuria

ANA +

pANCA- Arthrocentesis : 200 ml purulent liquid

Culture : + Staphylococcus aureus

Diagnosis : septic knee arthritis

1 week after 4th course of iv Cyclophosphamide ….

1 week before….ESR 15 mm/h,

CRP 2 mg/l

Normal C3, C4

Normal CBC

Palpable purpura?

? SLE vasculitis (possibile, low C3, C4, ANA +)

? Cryoglobulinaemia /viral infection

( HVC, HVB, HIV, cryoglobulines -)

? Drug induced vasculitis

(no recent medication added)

? Secondary vasculitis: S. Aureus infection

Hypersensitivity

vasculitis?

The first step in treating hypersensitivity

vasculitis is removal of the offending

antigen!

Staphylococcus aureus & vasculitis

Secondary vasculitis resulting from unusual pathologic

expressions of infections has been described

Associated with bacteriemia

Some authors sustaine that Saphylococcus aureus is

involved in induction and expression of ANCA-Associated

Small Vessel Vasculitis

1. Miranda-Filloy JA, Microscopic polyangiitis following recurrent Staphylococcus aureus bacteremia and

infectious endocarditis, Clin Exp Rheumatol. 2006 Nov-Dec;24(6):705-6.

2. Cees G. M. Kallenberg, Pathophysiology of ANCA-Associated Small Vessel Vasculitis, Curr Rheumatol

Rep. 2010 December; 12(6): 399–405.

Treatment:

Antibiotics (antibiogram) – 5 weeks

Vancomycin 15 mg/kg iv q12h

Ciprofloxacin 400 mg iv q 12h

Frequent joint drainage and lavage

Painkillers

Continues anticoagulant treatment

STOP:

Cyclophosphamyde iv

Cortisone pulses

Hydroxicloroquine 200 mg BID

Evolution…..

Acute monoarthritis

Paplable purpura

Normal ESR, CRP

Normal C3, C4

Normal CBC

ANA +

6 weeks

after…Now, 5 months after…

ESR 120 mm/h,

CRP 274 mg/l

WBC 15600/mm3

C3,C4 slightly low

ANA +

AB Plaquenil

Aceno

cumarolAceno

cumarol

Normal ESR, CRP

Normal C3, C4

Normal CBC

ANA +

Unclear facts….

? What was for sure the initial lesion:

- small vessel vasculitis lesion (secondary to

SLE)

- trombosis secondary to SAFL

? Why the CRP was positive at the biginning

? Surprisingly the disease remained stable after a

serious infection….