Case 1: 40-year-old African female with lupus flare Zahir Amoura

A 40-year-old African female was referred for fatigue, weight loss (-5 kg during the last month), fever (39°C) and right paralumbar pain. Physical exam displayed bilateral malar rash and there was no joint pain.

Laboratory tests revealed: Hb: 8.2 g/dL (12.0–16.0), white cell count (×10⁹/l): 2.00 (4.00–10.00), neutrophils: 0.952 (2.00–7.50), lymphocytes: 0.840 (1.50–4.00), platelets: 105 (×10⁹/l) (150–400). Creatininemia: 47 µmol/l (44- 80), ASAT: 188 (17–27), ALAT: 98 (11–26), Gamma-GT: 127 U/l (8–36), Total bilirubin: 12 µmol/l (2–17), albuminaemia: 23 g/l, fasting triglyceridaemia: 3.47 mmol/l (0.4–1.65); CRP: 177 mg/l (<5.00). Procalcitonin: 1.71 µg/l (<0.5), Fibrinogenaemia 4.1 g/l (2.0–4.0), positive ANA: 1/1280, positive anti-dsDNA: 84 UI, low C3: 0.61 g/l.

Blood cultures (X3): negative. Urine cultures: negative, daily proteinuria (g/24h): 2.5.

Kidney biopsy: Lupus nephritis Class III (A: 14%; C: 7%).

Thoracic-abdominal-pelvic CT scan: polyadenopathy: right and left sus-clavicular, axillar, mediastinal lymph nodes. Abscess of the right psoas.

Aspirate of the right psoas abscess displayed numerous Escherichia coli.

The patient was diagnosed with a concomitant lupus flare and a right abscess of the psoas.

Discussion Point

• Management of a severe infection concomitant with a lupus flare

Case 2: 54-year-old Caucasian female with SLE Zahir Amoura

A 54-year-old Caucasian female was diagnosed with systemic lupus erythematosus (SLE) in 2003 based on psychosis and lupus nephritis Class IV, positive antinuclear antibodies, positive dsDNA antibodies, low C3 level. She had been treated with prednisone, hydroxychloroquine and IV cyclophosphamide. Cyclophosphamide was switched to mycophenolate mofetil 2 g/day for 5 years. She had a renal relapse in 2015 (Class III + V) and was treated with mycophenolate mofetil (MMF) 2 g/day + prednisone 0.5 mg/kg/day). Prednisone was tapered to 5 mg/day and MMF was maintained. In January 2019, she had sudden changes of mood, becoming more irritable and sensitive. Her condition deteriorated over the next few days. She presented manic symptoms with psychomotor excitement, logorrhea, tachypsychia, euphoric state and insomnia. She had delusions and hallucinations with dysmorphophobic and nosophobic thematics. She was hospitalised and received olanzapine (40 mg/day), loxapine (50 mg/day) and clonazepam (3.5 mg/day). Cerebral spinal fluid analysis was normal. An electroencephalogram showed diffuse slow waves (0.5 to 1 wave per second). Antinuclear antibodies were positive (1/320), anti-DNA antibodies were negative; and C3 level was normal. A magnetic resonance imaging scan showed small and non-specific hyperintensities. She then became catatonic showing rigidity, mutism, staring, waxy flexibility and negativism. A diagnosis of SLE-induced psychosis with catatonia was made. Antipsychotic medications were stopped, and a high dosage of lorazepam (15 mg/day) was started together with three IV methylprednisolone pulses (1 g) followed by a month of 1 mg/kg/day oral prednisone, progressively tapered. MMF was stopped and switched to cyclophosphamide (0.7 g/m2; two weekly then monthly pulses) and 13 plasma exchanges. Catatonia as well as psychotic symptoms progressively improved.

In March 2019, she presented with fever (40°C), dyspnea with tachypnea (respiratory rate 28/min), supine blood pressure of 90/50 mmHg, tachycardia (105 BPM). Laboratory test were: Hb: 10.2 g/dL (12.0–16.0), neutrophils(×10⁹/l): 11.12 (2.00–7.50), lymphocytes: 0.260 (1.50–4.00), platelets: 355 (×10⁹/l) (150 – 400). Creatininemia: 150 µmol/l (44–80), ASAT: 88 (17–27), ALAT: 68 (11–26), Gamma-GT: 48 U/l (8–36), Albuminemia: 22 g/l,; C-reactive protein: 241 mg/l (<5.00). Procalcitonin 21 µg/l (<0.5), positive anti-dsDNA: 55 UI, normal C3:0.81 g/l

Arterial blood gas analysis demonstrated severe hypoxemia (PO2: 40 mmHg; PCO2 20 mmHg)

Chest CT scan displayed diffuse bilateral opacities. A diagnosis of Acute Respiratory Distress syndrome was done and the patient was refereed to Intensive care unit.

Discussion Point

• Management of a severe infection of pulmonary origin in SLE

Case 3: 19-year-old female (AM) with 1-month history of malar rash, polyarthritis, fatigue, fever David Isenberg

Patient AM presented aged 25 years with 6 month history of Raynaud’s and a 2 week history of malar rash, polyarthritis, fatigue and fever. On admission, her laboratory tests revealed ANA–1:640 and a positive anti dsDNA antibody. On Day 3, she had complained of proximal weakness and CK >3,000 iu/L. She was due to have a muscle biopsy on Day 7 but presented with severe abdominal pain and her amylase was >3,000 IU/L, she was presumed to have pancreatitis. Her condition deteriorated alarmingly between Days 8 and 10, when she developed adult respiratory distress syndrome and died.

Discussion Point

• What did the post-mortem show?

Case 4: 41-year-old patient (LC) returns from holiday in the Dominican Republic of Congo and presents with ↑+ proteinuria and fever David Isenberg

Patient LC had an Indian father and West Indian mother. At age 18 years she presented with arthritis and hair loss. Laboratory results revealed: postive ANA 1:2560; dsDNA, anti-RNP, ab and LAC. She was treated with steroids and azathioprine.

Aged 23 years, she went to see her GP, presenting with a cold blue fingers; 5 hours later she collapsed; several of her fingers were gangrenous. She was given prostacyclin, steroids, and cyclophosphamide and improved.

Age 31 years she presented with heavy proteinuria, biopsy revealed Class IV nephritis and she was given IV cyclophosphamide. For the following 8 years (age 32–40 years) she was managed on steroids and azathioprine, before stopping treatment. Aged 40 years, she experienced renal relapse which improved with prednisolone and azathioprine. Aged 41 years, she went on holiday to the Democratic Republic of the Congo, and returned with 4+ proteinuria, fever, and she felt unwell. She had a further renal biopsy.

Discussion Point

• What did the biopsy reveal?

Learning objectives

• Describe the causes of infections in SLE

• Discuss prevention of infections in SLE

• Explain management of difficult infections in SLE