21 results
Cutaneous and Systemic Findings in Dermatomyositis
Cutaneous Findings:
 - Heliotrope sign and edema
 - Nail-fold changes
 -
- Heliotrope sign ... or shawl sign ... - Gottron's sign ... - Interstitial lung ... Dermatomyositis #Diagnosis #Rheumatology
Autoimmune Myositis
Necrotizing myopathy is associated with higher CK levels, greater level of muscle weakness, less systemic
weakness, less systemic ... heliotrope rash, Shaw sign ... , holster sign, ... anti-synthetase syndrome ... Myositis #diagnosis #rheumatology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Clinical Features of Systemic ... effusion, Restrictive lung ... Demyelinating syndromes ... erythematosus #signs ... symptoms #diagnosis #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation Syndrome ... Periodic fever syndromes ... rash) • Heart, lung ... #Diagnosis #Management ... #Hematology #Rheumatology
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
female, asian • Systemic ... biliary, renal, lung ... increased IgG4+ cells ... Disease #diagnosis #management ... workup #treatment #rheumatology
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
• Chronic lung ... hemolytic anemia) • Systemic ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... hypogammaglobulinemia #immunology #hematology
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
vs Tumor Lysis Syndrome ... WBC >100k, + lab signs ... Lysis of tumor cells ... TLS #diagnosis #management ... #hematology
Chronic Lymphocytic Leukemia - Summary

Cancer affecting lymphocytes/Mature B cell neoplasm
 • Lymphocytes accumulate in large numbers
rendering the immune system ... Physical Exam/Signs ... ’s CLL Scoring system ... • Richter’s Syndrome ... workup #oncology #hematology
Blood Transfusion Reactions

Immune Mediated:
 • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
Blood Transfusion Reactions ... Related Acute Lung ... potassium from lysed cells ... Transfusion #diagnosis #management ... #hematology #reactions
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Central nervous system ... Nephrogenic DI Hematologic ... MALT Lymphoma • Lung ... #Rheumatology # ... Diagnosis #Management