Table 1 Initial presentation and autoantibodies in case 1 and 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.