A 42 yearold woman with chronic hemoptysis

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A 42 year-old woman with chronic hemoptysis

• Douglas B White, MD
• March 11, 2003

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• 42 year-old woman with a h/o MVR who presents
  with chronic hemoptysis.
• Patient was well until 10 months ago when she
  developed abnormal uterine bleeding that
  persisted for 2 months.
• 8 months PTA, she developed scant daily
  hemoptysis and fatigue.
• Evaluated by a cardiologist and found to have
  moderate MR and pulmonary HTN.
• Presented to SFVAMC for elective MVR.

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HPI (cont.)

• Patient endorses persistent cough with bright red
  brown sputum.
• Exercise tolerance lt 1 block

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• Medications
• Lasix 40 bid
• Lisinopril
• Ranitidine
• FESO4
• NKDA

• PMH
• MVR-1989
• Anemia
• Migraines
• Asthma
• CHB? DDD pacer
• Depression
• GERD

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• Social History
• 1ppd x 20years
• No IVDU-distant speed use
• Rare EtOH
• Lives in East Bay
• No travel

• Family Hisotry
• Unremarkable

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• Physical Exam
• 37.8 110/75 105 12 95 RA
• Obese woman in NAD
• Normal oropharynx
• Lungs CTA B
• CV lift, MR murmur
• Abdomen benign
• Trace LE edema
• Neuro normal

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Labs

• WBC 8
• Hct 31
• Platelets 114
• BUN 25
• Creat 2.0

• INR 1.5
• PTT 62
• UA negative

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10/02
Admission
10/02
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Admission CT chest
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• Echocardiogram
• -Normal LV size and function
• -Mildly thickened mitral valve with normal
  function trace TR
• -PAsp 65

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• Bronchoscopy
• -no endobronchial abnormalities
• -bacterial, fungal and AFB culture negative
• -PCP negative
• -many hemosiderin-laden macrophages and serial
  increase in bloodiness of BAL.

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Laboratory Data

• ANA 1180
• dsDNA negative
• Anti-GBM, C P-ANCA, anti-smith and anti-RNP
  negative.
• Normal C3, C4
• Pt became oliguric with creat 2.0? 3.6
• Repeat UA bland

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• Open lung biopsy
• Acute and chronic alveolar hemorrhage
• Numerous hemosiderin laden macrophages
• Pulmonary microthrombi
• Capillaritis

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Renal Biopsy- Microthrombi

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Laboratory Data

• Anti-cardiolipin IgG strongly positive
• Lupus anticoagulant positive.
• 5/01 from OSH
• Anticardiolipin IgG strongly positive
• Lupus anticoagulant strongly positive

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Antiphospholipid Syndrome

• Characterized by antibodies directed against
  phospholipids or proteins bound to phospholipids.
• Manifests as arterial venous thromboses,
  recurrent fetal loss, CVA and thrombocytopenia.
• Primary APS when it occurs alone secondary when
  it occurs with autoimmune disease.

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Four Types of Antiphospholipid Antibodies

• Antibodies resulting in false positive serologic
  test for syphilis. (Syphilis Ag embedded in
  cardiolipin).
• Lupus anticoagulants (causes prolonged PTT and
  rarely prolonged PT)
• Anticardiolipin Ab
• Anti- B2 glycoprotein Ab

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Disorders Associated with APS

• SLE (31 have LA, 23-47 with aCL)
• ITP (up to 30)
• Rheumatoid arthritis (7-50)
• Sjogrens Syndrome (25-42)
• Polymyalgia rheumatic, MCTD, Raynauds phenomenon.

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Infections, Drugs Antiphospholipid Ab

• Hepatitis A C, bacterial sepsis, mumps, HIV,
  syphilis, PCP, mononucleosis.
• Phenytoin, hydralazine, procainamide, quinine,
  propranolol
• Usually result in short-lived presence of
  anti-cardiolipin IgM.
• Possible association with increased thrombotic
  events.

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Clinical Manifestations of APS

• 1000 patients with APS from a European cohort.
• 82 female
• 53 primary APS
• 36 SLE
• 5 SLE-like syndrome
• 2.2 Primary Sjogrens syndrome
• 1.8 Rheumatoid arthritis
• 0.7 Systemic sclerosis
• 0.5 Dermatomyositis

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Presenting Manifestations of APS

• Deep Vein Thrombosis 32
• Thrombocytopenia (lt100k) 22
• Livedo Reticularis 20
• Stroke 13
• PE 13
• Fetal loss 8
• Hemolytic anemia 7
• MI 3

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Livedo reticularis in APS

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The Kidney in Antiphospholipid Syndrome

• Renal involvement occurs in 3 of APS.
• Mild proteinuria to ARF with active sediment.
• Renal pathology glomerular thrombosis, renal
  infarction, renal artery/vein thrombosis.
• Usually no glomerular inflammation.
• Presence of renal involvement does not change
  therapy.

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Pulmonary Manifestations of APS

• Pulmonary Embolism 14
• Pulmonary hypertension 2.2
• Pulmonary microthrombosis 1.5
• Fibrosing alveolitis 1.2
• Other (ARDS, DAH) 0.7

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Diffuse Alveolar Hemorrhage in APS

• 7 episodes of DAH in 5 patients with primary APS.
• Symptoms ranging from mild cough and fever with
  or without hemoptysis to respiratory failure.
• All were febrile and anemic (Hct lt31).
• Transbronchial and open lung biopsy revealed
  pulmonary microthrombi in all patients and
  capillaritis in half.
• All patients initially treated with high dose
  steroids cyclophosphamide if poor clinical
  response.

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Treatment of Antiphospholipid Syndrome

• Asymptomatic patients with aCL Ab or lupus
  anticoagulant should not be treated.
• Aspirin ineffective in preventing thrombotic
  complications of APS.
• Standard therapy is heparin? coumadin with goal
  INR 3.0.
• No compelling data supporting chronic steroids or
  immunosuppresive therapy.
• Steroids /- immunnosuppresives /-
  plasmapheresis recommended in catastrophic APS.

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Case Presentation

• The pt was initially treated with solumedrol 1g
  IV qd, cellcept and plasmapheresis with good
  clinical response.

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After steroids
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Case Presentation

• Given the resolution of radiographic
  abnormalities, the patient was started on
  anticoagulation.
• Several days into therapy, she became acutely
  SOB. V/Q scan showed acute PE.

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After coumadin
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References

• Mcneil et al. Immunology and clinical importance
  of antiphospholipid antibodies. Adv Immuno.
  199149193.
• Cervera et al. Clinical Immunologic
  manifestations of APS 1000 patients. Arthr
  Rheum. 2002461019-27.
• Espinosa et al. The lung in APS. Ann Rheum Dis.
  200261195-98.
• Gertner E. DAH in APS Spectrum of disease and
  treatment. J Rheumatol. 199926805-07.
• Khamashta et al. Management of thrombosis in APS.
  NEJM. 1995 332993.