Abstract :
Case Details: A 45 years hypertensive female presented with dry cough, dyspnoea and crusted non healing
ulcer on right ankle for one month. She had worsening anaemia, proteinuria and thrombocytopenia. CT
pulmonary angiogram showed pulmonary hypertension, left internal jugular vein thrombus with bilateral
multilobar opacities. Doppler USG showed right popliteal and left jugular vein thrombus. Serology
suggested low C3, C4, negative ANA with twice positive antiphospholipid antibodies. Bronchoalveolar
lavage was consistent with diffuse alveolar haemorrhage (DAH). Ulcer biopsy suggestive of lymphocytic
vasculitis consistent with immune complex deposition disease.
Fulfilling Systemic Lupus International Collaborating Clinics (SLICC) clinical and immunological criteria,
ANA negative SLE with DAH was diagnosed.
Conclusions: ANA negative SLE is a subgroup of SLE with similar clinical manifestations to their ANA
positive counterparts. Clinicians must be aware that ANA need not to present always in patients with
significant systemic disease with clinically suspected SLE.
Keyword :
Antinuclear antibodies, Antiphospholipid syndrome, Diffuse-alveolar haemorrhage, Systemic lupus erythematosus, Thrombocytopenia.