9 results
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
• Chronic lung ... lymphocytic interstitial lung ... and M panel Management ... DDX - Other causes ... #diagnosis #management
Wegener's granulomatosis
Wegener's is a collagen vascular disease with vasculitis involving the lung, kidney and sinuses.
In the
Wegener's granulomatosis ... collagen vascular disease ... vasculitis causes ... GPA #Wegeners #Granulomatosis ... Multifocal #Nodules #RadiologyAssistant
Pulmonary Renal Syndromes - OnePager Summary
Autoimmune ANCA vasculitis (AAV): GPA (granulomatous with polyangiitis), EGPA (eosinophilic granulomatosis
(eosinophilic granulomatosis ... life-threatening diseases ... Goodpasture's cause ... sparing of the lung ... differential #diagnosis #management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
highly inflammatory disease ... nervous system (CNS ... rash) • Heart, lung ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
Hypocomplementemia • Can cause ... • 8%-27% for CNS ... MALT Lymphoma • Lung ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
(depending on CNS ... steroids • In mild ... or autoimmune disease ... HLH #Treatment #management ... #hematology
Autoimmune Myositis - Differential Diagnosis Framework

Inflammatory Myopathies: Commonly symmetric proximal muscle weakness, no substantial muscle pain
Anti-MDA5 (RP-ILD ... ), dermato-rheumatologic ... nucleotidase 1A (Anti cN ... statins - probably caused ... Myositis #Myopathy #rheumatology
Langerhans Cell Histiocytosis (LCH)

Granulomatous Lesions comprising langerin-positive histiocytes and an inflammatory infiltrate can arise in virtually
Histiocytosis (LCH) Granulomatous ... for bone, skin, lungs ... Incidence: 4–5 cases ... CNS involvement: ... Histiocytosis #Diagnosis #Management
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
Muscle atrophy may cause ... had more severe disease ... Non-small-cell lung ... Dermatomyositis #diagnosis #management ... #rheumatology #