SLE Presenting with Hemoptysis

Today Dr. Bien presented a very interesting case of a young man presenting with hemoptysis and acute respiratory failure in the setting of a month course of fevers, malaise and weight loss. In addition to hemoptysis, exam was notable for developing alopecia, ulcers on the hard palate and a S3. Labs were noted pancytopenia, elevated LDH, elevated CK and hematuria. After exploring the differential for hemoptysis, rheumatologic disorders were highest on the differential. However, his initial ANA was negative. On repeat he had a floridly positive ANA, ant-dsDNA and very low complements (C3 and C4) consistent with systemic lupus erythematosis (SLE) complicated by diffuse alveolar hemorrage (DAH) and myocarditis. The Prozone Effect was felt to be the reason why the initial ANA was negative.

CT scan with lobular GGOs and "crazy paving" concerning for DAH as referenced in the recent case report from  Respiratory Medicine . 

CT scan with lobular GGOs and "crazy paving" concerning for DAH as referenced in the recent case report from Respiratory Medicine

1) Hemoptysis can be streaks of blood to gross blood. Massive hemoptysis is defined as 100-600ml of blood within 24 hours. Once you determine that the hemoptysis is coming from the airway (not the mouth, stomach, etc) the differential can be built around the location with a few additions.

  • Airway: bronchitis, bronchiectasis (CF), emphysema, fistula formation, bronchial adenocarcinoma, Dieulofoy lesion, metastasis
  • Pulmonary Parenchyma:
    • Infectious: anthrax, abscess, fungus, parasite (strongyloides), TB, tuleremia, virual (HSV, flu), yersinia
    • Rheumatologic: amyloid, anti-GBM (formally Goodpasture's), Behcet's, collagen vascular disease (Ehlers-Danlos), granulomatosis with polyangiitis formally Wegener's), APLA, SLE
  • Vascular: congential heart disease, heart failure, mitral stenosis, hereditary hemorrhagic telangiectasia, tricuspid endocarditis, pulmonary AVMs, PE with or without infarction, pulmonary veno-occlusive disease
  • Drugs/Toxins: bevalizumab, cocaine, nitrogen dioxide
  • Misc: celiac with idiopathic pulmonary hemosiderosis, catamenial hemoptysis with menses and endometriosis
  • Trauma: blunt force, ETT placement, bronch etc.
  • Coagulation Disorders: DIC, TTP, HUS, vWD

2) ANA is 93-95% sensitive and 57% specific. However, the Prozone Effect is a false negative response resulting from high antibody titer which interferes with the formation of antigen-antibody formation necessary for the positive ELISA.

prozone effect.png

3) DAH is a rare and life threatening complication of SLE. It is traditionally managed with high dose IV corticosteroids first line. Cyclophosphomide use has been associated with improved survival. Plasmapheresis on the other hand shows no difference. Use of Rituximab is up and coming with case reports. For additional reading, see the systematic review from 2015 in the Journal of Clinical Rheumatology and the recent case report in Respiratory Medicine using rituximab.