Lupus!! (Yay!!!…..???)

Goals

Outline The Basics Emergency Stuff –Presenting symptoms –Complications –Infection Quizzes… with prizes!

Definition “Multi-system autoimmune connective tissue disorder with a broad range of clinical presentations”

Epidemiology Varies among ethnic groups –Which is most commonly affected? Age Distribution (2-80) –Highest incidence age group? Women > Men Incidence has tripled since the 1970’s –Why?

Etiology Genetic –Up to 57% concordanc e –Up to 12% of relatives Environmental - Quiz Question

Quiz 1 Which of the following has not been shown to exacerbate lupus? a) sun exposure b) smoking c) Alcohol d) Sulfa medications e) Pregnancy f) High-dose Estrogen

Clinical Features Rheumatologic Dermatologic Renal Neurologic Cardiac Pulmonary Gastrointestinal Hematologic Dermatologic…

Quiz 2 What is the difference between Raynauds disease and Raynauds Syndrome?

Diagnosis Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis Serositis (pleuritis, pericarditis) Renal disorders Neurologic disorders (seizure, psychosis) Hematologic disorders (anemia, leukopenia, lymphopenia, thrombocytopenia) Immunologic disorders (anti-dna, anti-Sm, aps) ANA

Quiz 3 Which of the following is not true concerning a butterfly rash? a)Also known as a malar rash b)Not pathognomonic for Lupus c)Red/purplish in colour with sharp edges d)Not itchy e)Involves the naso-labial folds

Treatment Avoidance of environmental factors Anti-malarials (hydroxychloroquine) Salicylates or NSAIDS Glucocorticoids (prednisone, methylprednisolone) Immunosuppresives/Cytotoxic (cyclophosphamide, azathioprine) BMT, IgG, biologics, many others…

What we really care about… How to recognize a first presentation of Lupus (less important for us) Common complications of disease Common complications of treatment

How does Lupus usually present?

First Presentation Non-specific (fever, wgt loss, fatigue, anemia) Photosensitive rash Arthralgia or arthiritis Raynaud Phenomenon Serositis Nephritis/Nephrotic Syndrome Neurologic symptoms Alopecia Phlebitis Recurrent abortion

The Obvious…

The Not So Obvious…

Take home points (THP’s) Fatigue!! Skin/arthritis/fever Young females Any pt presenting with multi-system complaints Recurrence of symptoms

Mortality

SLE (21-34%) Infection (22-25%) CVS (16-42%) Thrombosis (up to 26%) Cancer (6-12%)

Complications Cardiac Gastrointestinal Neurologic Pulmonary Renal Hematologic Others

Cardiac Pericardial (up to 55%) Myocardial (up to 78%) Valvular (over 50%) Coronary arteries (up to 16%)

Question 2 IC deposition causes recurrent inflammation of leaflets causing varying degrees of scarring, fibrosis, etc…

Valvular The prevalence of infective endocarditis is comparable to those with mechanical heart valves The incidence of ischemic stroke in SLE is 10-20% % of these have confirmed valvular thrombi Mortality in SLE patients with cardiac disease is 20% over 5 years

Pericardial Most frequent cause of symptomatic disease Large effusions/complications are rare

Myocarditis Uncommon and usually asymptomatic ST and T wave changes +/- symptoms of heart failure

Coronary Artery Disease Independent RF More than 5 times the baseline risk Traditional RF’s also more prevalent Glucocorticoids

Neuropsychiatric Stroke Seizure Headache Neuropathy *Psychosis Others (meningitis, cranial neuropathies, transverse myelitis)

Stroke Reported in up to 19% of patients Another study: risk of death doubled compared to non-SLE controls Most occur early in course (first 5 years) Recurrent strokes are common

Seizures 10-20% of patients Generalized and Partial

Pulmonary Pleuritis URTI Acute/chronic pneumonitis Pulmonary hypertension Pulmonary Hemorrhage

Other Complications Renal –Nephrotic syndrome Gastrointestinal –Intestinal Vasculitis Hematologic –Thrombocytopenia, anemia

Complications of Tx Steroids –Diabetes –Osteroporosis –Weight gain –Pancreatitis –Osteonecrosis –Atherosclerosis –…

Complications Infection –Studies reveal more than 50% of fever in SLE patients are due to active disease However, at some point in the course of their disease over half of SLE patients will have serious infections.

Infection RF’s for infection include active lung or renal disease, neutropenia, leukopenia, steroid use <3 months ago, dose of steroids Anti-malarials protective *most RF’s are LR’s <2 except for lung involvement

Question 5 Where are Lupus patients most likely to get infections? a)Lungs b)Urine c)Skin d)CNS e)Abdomen

Infection Pneumonia (40), bacteremia (25), cellulitis (10) Other (25) - pyelo, abdominal, Tb, meningitis, osteomyelitis, etc

Infection Most likely Gram -, gram + cocci Causal Agents: S. aureas, S. Pneumo, Salmonella, E. Coli, Klebsiella, Pseudomonas… Rarely fungal, viral, anaerobes

Take Home Points Diagnosis –Not our first priority, but… –Know the classic but don’t expect it! –“RF’s” - young female, presenting with multi-system complaints –Fatigue

Take Home Points Complications –Cardiac –Neuro –Pulm –Other

Take Home points Infection –RF’s –Tx fever as infection until proven otherwise –Common bugs but variable –Treat as we would normally treat