Our patient is a 24-year old caucasian male diagnosed with SLE and associated antiphospholipid syndrome (APS) in 2001 at the age of 9. Initial symptoms were arthralgia, livedo reticularis and a decrease in complement. In 2005 he developed a class 4a glomerulonephritis. He was treated with prednisolone and cyclophosphamide as induction therapy and with azathioprine as maintenance treatment. Azathioprin was later changed to mycophenolate mofetil (MMF). His renal function normalized with an estimated glomerular filtration rate (eGFr) of 71 ml/min but permanent albuminuria of 2-3g/24 h. The diagnosis of APS was made in 2009 based on deep vein thrombosis, positive lupus anticoagulant >150 s (<42 s), elevated beta2-glycoprotein IgM 51 103 units/l (<20 units/l) and beta2-glycoprotein IgG 75 103 units/l (<20 units/l) and an increased APTT of 138 s (25-38 s) and he was therefore treated with warfarin (INR 2.0-3.5).
In spite of the heavy immunosuppressive treatment, his SLE worsened. In 2008, he was seen in our department for the first time due to an increased cough, dyspnea and hemoptysis. He was in a physical good shape playing football in spite of his kidney and lung symptoms even when his haemoptysis worsened. A high-resolution computed tomography (HRCT) showed lobular ground glass attenuation compatible with alveolar bleeding (Fig. 1), confirmed by a bronchoscopy showing hemorrhagic lavage fluid and hemosiderin-laden macrophages (Fig. 2). Culture was without mycobacteria or other microorganisms. During the DAH episode he had low C3c 5.5 mumol/l (6.7-13.4 mumol/l), thrombocytopenia 84 109/l (145-350 109/l), low hemoglobin 6.8 mmol/l (8.3-10.5 mmol/l) and a hematocrite of 0.37 (0.40-0.50). The findings were interpreted as an activation of his SLE and the mycophenolate dosage was increased. Due to recurrent haemoptysis, RTX was added to his treatment. As induction therapy, 1g of RTX was administered twice two weeks apart. His condition improved rapidly. Forced expiratory volume in 1 second (FEV1) increased from 2.47L to 2.93L and forced vital capacity (FVC) increased from 2.72L to 3.28L. His haemoptysis decreased significantly. HRCT show significantly regression in ground glass attenuation (Fig. 3).
However, after 3 months, the dyspnea and haemoptysis returned and he again required RTX followed by continuous RTX 1g initially once a year. Due to recurrent and increasing hemoptysis after two years of treatment, the intervals were shortened to 6 months between each series of RTX.
During the 8 years with DAH, the patient was only hospitalized once due to a pulmonary infection. He has never been hospitalized due to DAH and has never needed mechanical ventilation.
His current treatment consists of prednisolone 10 mg x 1, mycophenolate mofetil 500 mg x 3, hydroxychloroquin 200 mg x 1 and 1 g of RTX every 4 months.
High-resolution computed tomography (HRCT) showing lobular ground-glass opacities and in some areas interlobular septal thickening superimposed on ground-glass opacity (crazy paving pattern) compatible with alveolar bleeding.