Belimumab is started with the resolution of serositis
and the clinical and analytical improvement of SLE markers.
Macrophage activation syndrome (MAS) is a severe complication of rheumatic diseases, most frequently systemic juvenile idiopathic arthritis (sJIA) - a rare systemic disorder of auto-inflammatory nature with common clinical manifestations such as daily spiking fever, typical transient cutaneous rash, arthritis, lymphadenopathy, hepatosplenomegaly and serositis
. MAS is characterised by fever, hepatosplenomegaly, liver dysfunction, cytopenias, coagulation abnormalities and hyperferritinaemia, possibly progressing to multiple organ failure and death.
* The 11 criteria are as follows: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis
(pericarditis or pleuritis), renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and antinuclear antibody.
SLE patients with infections showed significantly a higher incidence rate of fever (84.6 vs 28.2%) and serositis
(84.6 vs 23.1%) compared with non-infection patients (both P < 0.05).
(2) Furthermore, based on the American College of Rheumatology's Systemic Lupus International Collaborating Clinic (SLICC) score, the patient also met criteria for SLE with at least four criteria (ANA, leucopenia, serositis
as per pleural effusion, and low complement).
: review of its clinical features, pathophysiology and management strategies.
, oral ulcers and arthritis etc.
Clinical signs included photosensitivity malar rash, discoid rash, oral ulcers, arthritis, serositis
, renal involvement, central nervous system involvement, fever Raynaud's phenomenon, and interstitial lung disease.
For example, acute cutaneous manifestations received 16 points, serositis
9, oral ulcers 16, thrombocytopenia 15, and so forth.
A typical episode reveals itself with fever and serositis
. The skin and muscle involvement are not common in a typical episode.
The clinical data included the initial presentation to the nephrologist and symptoms of SLE (photosensitivity, arthritis, oral ulcers, serositis
, and malar, renal, cerebral, hematological and discoid rashes).