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Table 1 Initial presentation and autoantibodies in case 1 and 2

From: Anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis: two case reports and review of literature

  Case 1 Case 2
Initial symptoms Arthralgia, abdominal pain, cough, facial tenderness. Cough, arthralgia, morning stiffness, depression, sicca syndrome.
Initial clinical findings Four tender and swollen finger joints (not better described), axillary lymphadenopathy left, tenderness in the left iliac fossa. No signs of neuropathy. Tender right 3rd MCP joint and right shoulder, erythema right lower limb. No signs of neuropathy.
Histology Necrotizing vasculitis in tissue from the intestinal resection. No signs of vasculitis and no granulomas in the collected sinus tissue. Transthoracic biopsy: lymphohistiocytic infiltrate with central fibrinoid necrosis, no granulomas. Transbronchial biopsy: lymphogranulocytic infiltrate, no signs of vasculitis.
Initial laboratory values CRP, ESR, cell counts and creatinine: within normal range, immunoelectrophoresis with modest hypogammaglobulinemia (IgG, IgA), proteinuria and cryoglubulins negative. ESR 24mm, leucocytes 3000/mm3, CRP 20mg/l (<5), creatinine, C3, C4 within normal range, normal immunoelectrophoresis, Anticardiolipin antibodies and cryoglubulins negative, urine analysis without proteinuria.
Autoantibodies when first tested in our clinic ANA 1:160, Anti-dsDNA negative, Anti-ENA negative, RF negative, ACPA 99U/ml (<20), ANCA 1:800, Anti-PR3 negative, Anti-MPO negative. ANA 1:320, Anti-dsDNA 75U/ml (<55), Anti-ENA negative, RF 63U/ml (<15), ACPA negative, ANCA 1:500, Anti-PR3 negative, Anti-MPO negative, lupus anticoagulant antibodies negative.
Initial investigations Abdominal scan: absence of lymphadenopathy, no signs of diverticulitis. Radiography: normal skeletal imaging of hands and feet, normal chest radiography.
Chest scan: 4mm nodule superior lobe left. Schirmer Test negative.
Sinus scan: signs of chronic maxillary bilateral sinusitis. Salivary gland biopsy: modest lymphocytic infiltration (Focus score <1).
Lung function: signs of obstruction.
Computer tomography: four pulmonary nodules.
Treatments Prior to 2007: Gold salt, Mycophenolate mofetil, Etanercept, MTX. 2000: Leflunomide, later MTX
4/2007: Cyclophosphamide 2001-2002: Azathioprine.
8/2007: Etanercept, later Adalimumab. 2/2002-8/2002: Cyclophosphamide.
8/2008: Rituximab, MTX. 8/2002: Infliximab, MTX.
1/2010: Abatacept, MTX. 4/2003: Etanercept, MTX.
4/2010: Tocilizumab, MTX. 10/2004: Rituximab.
  1. MCP: metacarpophalangeal, CRP: C-reactive protein, ESR: erythrocyte sedimentation rate ANA: antinuclear antibody (Titre), dsDNA: double-stranded DNA, ENA: Extractable nuclear antigen, ANCA: anti-neutrophil cytoplasmic antibodies (Titre), PR3: proteinase 3, MPO: myeloperoxidase, ACPA: Anti-cyclic citrullinated protein antibody, RF: rheumatoid factor, MTX: Methotrexate, Ig: Immunoglobulin.