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
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.
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 +
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….
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