9 results
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Adult-Onset Still's Disease ... Clinical triad ... decreased glycosylated fraction ... #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 ... biliary, renal, lung ... #diagnosis #management ... #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
rash) • Heart, lung ... thrombocytopenia • Liver function ... Treatment: • Corticosteroids ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Portopulmonary Hypertension
Presence of pulmonary arterial hypertension in a patient with portal hypertension
No other obvious causes (chronic
causes (chronic lung ... disease, left heart ... Transthoracic echocardiogram ... pressure < 15mmHg TREATMENT ... Hypertension #diagnosis #management
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Pulmonary Thromboendarterectomy
Chronic Thromboembolic Pulmonary Hypertension
Definition: mean pulmonary-artery pressure > 25mmHg
Plexiform-lesion formation Clinical ... Ventilation-perfusion lung ... Bypass Other Treatment ... have inoperable disease ... Thromboendarterectomy #diagnosis #management
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
salivary ducts Clinical ... MALT Lymphoma • Lung ... Adenocarcinoma Treatment ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Pseudogout Summary - Calcium Pyrophosphate Deposition Disease (CPPD)

Pathophysiology:
Pyrophosphate produced by chondrocytes likely precipitates with calcium to
pain, loss of function ... Acute Pseudogout: Treatment ... ≥2 attacks • Long-term ... #Rheumatology # ... diagnosis #management
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
auricular papules Clinical ... had more severe disease ... and diminished functional ... positive patients Treatment ... #rheumatology #
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 ... nails, bronchi, lungs ... Fungal #Diagnosis #Management