10 results
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
Somatic) Syndrome Clinical ... thromboembolic disease ... • giant cell arteritis ... myeloid cells NEJM ... #Somatic #rheumatology
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Clinical triad ... Fever, Arthralgia/arthritis ... : other autoinflammatory ... #diagnosis #management ... #treatment #rheumatology
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... Clinical history ... concentration PET-CT ... #diagnosis #management ... workup #treatment #rheumatology
Giant cell arteritis (GCA)

Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults
Diagnosis = clinical ... in literature (NEJM ... cases (Cornelia NEJM ... them, but urgent rheumatology ... Symptoms #Diagnosis #Management
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
Etiology unknown: Autoinflammatory ... nonerosive inflammatory arthritis ... osteoarticular and skin disease ... SAPHO #Syndrome #Rheumatology ... #diagnosis #management
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... • Chronic lung ... Chest CT: • Ground-glass ... and M panel Management ... #diagnosis #management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
fever syndromes Clinical ... Fever is the main clinical ... rash) • Heart, lung ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Squamous-Cell Carcinoma of the Lung- ...Physical examination revealed decreased air entry in the base of the
Carcinoma of the Lung ... base of the right lung ... indicated stage IV disease ... #NEJM #clinical ... #radiology #CT #
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
salivary ducts Clinical ... Arthralgia and Arthritis ... MALT Lymphoma • Lung ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Mycoses

HISTOPLASMOSIS
• Inhalation of conidia → Yeast → travel to lymph nodes → spread in body
• Bird
pulmonary symptoms • Rheumatologic ... has five major clinical ... change in the lungs ... Meningitis, septic arthritis ... Fungal #Diagnosis #Management