Case of Macrophage activation syndrome, rare complication of Sjögren’s syndrome: a report

Dr B. S. Kane at Department of Internal Medicine, Cheikh Anta DIOP University, Dakar, Senegal and colleagues have reported a case of Macrophage activation syndrome, a rare complication of primary Sjögren’s syndrome. The case has appeared in the Journal of Medical Case Reports.

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disease that occurs more frequently in individuals with systemic juvenile idiopathic arthritis (SJIA) and in those with adult-onset Still disease. The association of macrophage activation syndrome and primary Sjögren’s syndrome has been rarely reported in the literature.

A 26-year-old Mauritanian and Berber woman was followed for 3 years for primary SS. This diagnosis was made on the basis of the following factors: dry eye syndrome, chronic non-erosive peripheral arthritis, positive Schirmer’s test, anti-SSA > 8 IU antibodies, and chronic lymphocyte grade IV sialadenitis (based on Chisholm and Mason classification). The remainder of the immunological assessment showed a titer of antinuclear antibodies at 1:80, and autoantibodies to double-stranded deoxyribonucleic acid (anti-dsDNA antibodies), rheumatoid factor, and anti-citrullinated peptide antibodies (ACPA) were negative.

This autoimmune disease was treated with prednisone (5 mg daily), hydroxychloroquine (400 mg daily), and methotrexate (15 mg weekly). Three months after stopping her disease-modifying antirheumatic drugs (DMARD), she was hospitalized for acute fever and inflammatory arthralgia.

At admission, she had a temperature of 39.5 °C, heart rate of 120 beats/minute, blood pressure of 110/80 mmHg, and breathing rate of 22 cycles/minute. A musculoskeletal examination showed synovitis of her wrists and knees. A pleuropulmonary examination revealed asymmetric crackling rales at the pulmonary bases. The activity of the disease was evaluated at 35 on the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI). The rest of the clinical examination was within normal limits; notably, there was no hepatosplenomegaly or lymphadenopathy.

The laboratory tests showed a bicytopenia with the biological inflammatory syndrome and hyperferritinemia. We summarized the laboratory data of our patient during her last visit before the loss of follow-up and during the hospitalization in. A bone marrow aspiration showed hyperplasia of the granular lineage with plasmacytosis and images of hemophagocytosis. At this stage the diagnosis of MAS was retained with an H-Score of 219 points and a diagnostic probability of 93–96%

Screening for infections: the Plasmodium thick blood test, blood cultures, cytobacteriological examination of urine, cytobacteriological examination of sputum, acid-fast bacillus (AFB) search (for Mycobacterium tuberculosis), Epstein–Barr virus (EBV) polymerase chain reaction (PCR), and human immunodeficiency virus (HIV) serology were all negative. Standard radiography revealed interstitial syndrome at the pulmonary bases of the thorax. Thoracic computed tomography showed non-specific interstitial lung disease. The diagnosis of primary SS complicated by interstitial lung disease and MAS was retained.

Initial treatment was based on an increase in corticosteroid therapy to 1 mg/kg per day with blood transfusion. The outcome of her clinical condition, after 1 week of treatment, was marked by the persistence of intermittent fever with peaks at 39–40 °C. Etoposide treatment was initiated at a rate of 150 mg/m2 (200 mg in a single intravenous injection). Her clinical course was marked by a clear improvement in the symptomatology, with stable apyrexia, a C-reactive control protein of 13.7 mg/l, and a hemoglobin level of 8.2 g/dl obtained after the first 24 hours (Fig. 1). On discharge, she was switched to Imurel (azathioprine; 100 mg/day) and hydroxychloroquine (400 mg/day) combined with corticosteroid therapy. A follow-up 2 months later, with good adherence and tolerance of the treatment (patient self-assessment), showed complete regression of the cytopenia, a negative C-reactive protein, and serum ferritin at 224 μg/l.