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					ICM
NILAM PATEL

Case History
A 37yr old lady, mother of 2 children attended the OPD with complaints of left sided chest pain and breathing difficulty of 3 days duration. The pain is pricking type which is more on inspiration. She also gives h/o recurrent episodes of pain in the small joints of hands and feet for which she was on treatment with Prednisolone. On examination patient had pallor+, vitals- stable. She had bilateral spindling of joints and hands- more so in metacarpophalengeal and proximal interphalengeal joints. Systemic examination revealed decrease breath sounds in the left axillary area. Other systems- WNL

Investigations

Results: Hb= 11% TLC= 12000 DLC= P36, L63, Eo1 ESR= 80mm/1sthr Chest X-ray= dense opaque area in the Lt lower zone

Normal Values 12-16 G/dl 4000-10,o00/cmm

Interpretation Low Low Low High

P60, L35, E3-5
Upto 20mm/hr

Questions
 What is your likely diagnosis?
 What other physical signs will you look for in this

patient?  What other investigations will you order in this patient?

History Importance
 A 37yr old lady, mother of 2 children  Rheumatoid Arthritis  Gynecological Disorders  Diabetes Mellitus  SLE  Hypertension  Anemia

Complaints
 Complains of left sided chest pain and breathing

difficulty of 3 days duration. The pain is pricking type which is more on inspiration.


Pleurisy

Complaints
 Recurrent episodes of pain in the small joints of

hands and feet for which she was on treatment with Prednisolone.


Chronic disorder--- CTD

Examination
 Pallor +  Anemia of chronic disease  Steroids
 Vitals were stable

Examination
 Bilateral spindling of joints and hands- more so in

metacarpophalengeal and proximal interphalengeal joints.

Examination
 Systemic examination revealed decrease breath

sounds in the left axillary area
 

Underlying pleural effusion History is insufficient; does not provide percussion results where a stony dullness is elicited when there is pleural effusion

Differential Diagnosis
 Rheumatoid Arthritis
 Ankylosing Spondylitis  SLE  Reiters Syndrome  RA + conjunctivitis + urethritis

Rule Out
 Rheumatoid Arthritis  No morning stiffness  No mention of RF finding  No swan neck deformity  Ankylosing Spondylitis  Affects mainly axial vertebrae  Prevalence more in male than female
 Reiters Disease

Diagnosis
Most likely to be Systemic Lupus Erythromatosus

Criteria for Diagnosis of SLE
1.

Malar rash:


Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

2.

Discoid rash:


Photosensitivity 4. Oral ulcers:
3.


usually painless Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion

5.

Arthritis:


6. Serositis:  Pleuritis— convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion OR  Pericarditis--documented by ECG or rub or evidence of pericardial effusion 7. Renal disorder  Persistent proteinuria greater than 0.5 grams per day or grater than 3+ if quantitation not performed OR  Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed 8. Neurologic disorder  Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance OR  Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

9. Hematologic disorder  Hemolytic anemia--with reticulocytosis OR  Leukopenia--less than 4,000/mm  Lymphopenia--less than 1,500/mm OR  Thrombocytopenia--less than 100,000/mm, in the absence of offending drugs 10. Immunologic disorder  Anti-dsDNA, anti-Sm, and/or antiphospholipid 11. Antinuclear antibody  An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with "drug-induced lupus" syndrome

Etiology
SLE results from tissue damage caused by pathogenic subsets of autoantibodies and immune complexes

Abnormal immune response includes: 1. Polyclonal and antigen specific T and B lymphocyte hyperactivity 2. Inadequate regulation of this hyperactivity This response depends upon interactions of susceptibility genes and environment

Pathogenesis
 T cell help is critical in the development of full blown

disease  Abnormal immune response  sustained production of autoantibodies  immune complexes:

 

Anti-DNA: can bind to tissue and cause complement + Subsets of anti-DNA and anti-RNP: can bind and enter living cells, altering their function Other autoantibodies can cause damage by direct binding to cell membranes, that cause those cells to be phagocytized and destroyed.

 Structure antigens that stimulate autoantibody

production

Clinical Manifestations
 May involve one organ system or maybe multisystemic  Autoantibodies are detectable at onset of disease  Severity varies from mild and intermittent to persistent and

fulminant  Most patients experience exacerbations interspersed with periods of relative quiescence  Remissions with no symptoms requiring no therapy occur in up to 20% patients  Systemic symptoms:



  

Fatigue Malaise Fever Anorexia Weight loss

Clinical Manifestations by System

 Musculoskeletal  Cutaneous  Renal  Nervous system  Hematologic  Cardiopulmonary  GIT  Ocular

Musculoskeletal System
 Most patients experience arthralgia and myalgias 

develop intermittent arthritis  Physical Finding:


Symmetric fusiform swelling in joints, most commonly PIP and MCP joints of hands, wrists, and knees

10% of pts develop swan-neck deformities of fingers and ulnar drift at MCP joints

Cutaneous Manifestation

Cutaneous Manifestations
 Malar ―butterfly‖ rash  Photosensitive  Fixed erythematous rash  Flat or raised  Over cheeks and bridge of rash  Often involves chin and ears
 Scarring is absent  An diffuse maculopapular rash predominant in sun

exposed areas indicates disease flare  Loss of scalp hair – extensive and patchy; can regrow

Cutaneous Manifestations

Renal Manifestations
 Igs deposited in glomeruli  but only half have

clinical nephritis which is defined by proteinuria.  Early in disease most pts are asymptomatic  Some develop edema and nephrotic syndrome. Urinalysis:
 



Hematuria Cylindruria Proteinuria

Nervous System
 Any region of brain can be involved, meninges, spinal cord,

cranial and peripheral nerves  Mild cognitive dysfunction is the most frequent manifestation. Can also get headaches and seizures.  Less frequent, can get psychosis and acute confusional state  Lab diagnosis is difficult
 

About 70% pts have abn. Electroencephalograms- show diffuse slowing or focal abnormalities CSF shows:
 

Elevated protein levels in 50% pts Increased mononuclear cells in 30%

 

To detect acute and chronic lesions  MRI CT to rule out hemorrhage or mass lesions

 Neurologic problems usually improve with

immunosuppressive therapy- reoccurrences seen in 1/3 of patients.  Due to the years of exposure of blood vessels to circulating immune complexes and hyperlipidemia from glucocorticoid therapy predisposes the patient to degenerative cerebral and coronary artery disease

Hematologic Manifestation

Hematologic Manifestation
 Anemia of chronic disease
 Hemolysis occurs in a small proportion of those with

a positive coombs test  usually responds well to high dose glucocorticoid therapy  Leukopenia  Mild thrombocytopenia  LA is a antiphospholipid Ab  recognized by prolongation of aPTT and failure of added normal plasma to correct prolongation

Cardiopulmonary System
 Pericarditis is the most common manifestation
 Effusions can occur  tamponade  Myocarditis  arrythmias  sudden death, and/or

heart failure  Valvular insufficiency  MI

Cardiopulmonary System
 Pleurisy and pleural effusions are common
 Lupus pneumonitis  Fever  Dyspneoa  Cough  Most common cause of pulmonary infiltrates in pts

with SLE is infection  ARDS is rare but has a high mortality rate

GIT
 Nausea, diarrhea, vague discomfort
 Vasculitis of intestine is most dangerous Crampy abdominal pain  Vomiting  Diarrhea  Acute pancreatitis- raised serum amylase  Use glucocorticoid therapy

Ocular Manifestation
 Retinal vasculitis  few days  blindness
 Need aggressive immunosuppression  Examination shows areas of sheathed, narrow retinal

arterioles and while exudates (cytoid bodies) adjacent to vessels

Laboratory Diagnosis
Non-specific:  ANA- best screening test
Specific:  Anti-dsDNA and Sm Disease activity  High serum ANA and anti-dsDNA  Low levels of complement

Hematologic parameters Urinalysis Serum creatinine levels

Drug Induced SLE
Cause SLE like syndrome Drugs:  Procainamide  Hydralazine  Isoniazid  Chlorpromazine  D-penicillinamine  Practolol  Methyldopa  Quinidine  Interferon alpha

Drug Induced SLE
 Genetic predisposition  Determined by drug acetylation rates  Procainamide  Induces ANA in 50-75% of individuals w.in a few months  Hydralazine  Induces ANA in 25-30%  Most common are systemic complaints and

arthralgias, polyarthritis and pleuropericarditis  Have ANA and antibodies to histone  Ab to dsDNA and hypocomplementemia are raredistinguish bw idiopathic and drug induced SLE

Prognosis
 Survival in pts is:
   

90-95% at 2yrs 82-90% at 5yrs 71-80% at 10yrs 63-75% at 20yrs High serum creatinine levels Hypertension Nephrotic syndrome Anemia Hypoalbuminemia and hypocomplementemia at time of diagnosis Low socio economic status Infections and active SLE Thromboembolic events

 Poor prognostic factors:
 


  

 Leading cause of death in first decade:


 Leading cause of death in second decade:


What other physical signs will you look for in this patient?
 Butterfly rash
 Alopecia  Oral/nasal ulcers  Vasculitic lesions  Adenopathy pleural/pericardial/peritoneal signs  Edema  Neurological deficits

What other investigations will you order in this patient?
 ANA- Antinuclear antibody test
 Urine Analysis  Protein  RBC  Casts  Serology  Antibodies to dsDNA  Sm SSA(Ro)  RNP RBC (coombs)  Phospholipids- recurrent abortions in females  Complement


				
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posted:9/29/2008
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