8 results
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation ... Systemic lupus erythematosus ... nervous system (CNS ... Treatment: • Corticosteroids ... #Management #Hematology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Systemic Lupus Erythematosus ... Demyelinating Syndrome ... Cerebrovascular Disease ... Puncture, EEG Treatment ... #rheumatology #
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Malignancy (e.g. hematologic ... Shrinking lung syndrome ... involve PNS and CNS ... vein thrombosis Hematologic ... Chronic B-cell activation
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
basal ganglia, CNS ... meningitis), MCC CNS ... Arthritis, AS Treatment ... diagnosis #management #signs ... #symptoms #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... excessive macrophage activation ... age, however the disease ... Diagnosis: HLH signs ... #hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
ischemia - Skin: erythematous ... • Cogan Syndrome ... systemic lupus erythematosus ... purpura: strong sign ... • ANA (Suggest CTD
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
purpura: Strong sign ... ischemia • Skin: Erythematous ... Mucocutaneous lymph node syndrome ... vasculitis • Behcet syndrome ... vasculitis • Cogan syndrome
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
subcutaneous edema Pts ... chest (in a V-sign ... shoulders (in a shawl sign ... had more severe disease ... positive patients Treatment