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LP-167 Uncommon initial presentations of SLE – a case series
  1. Madusha Jayasinghe and
  2. Duminda Abeysinghe
  1. Rheumatology, Rheumatology and Rehabilitation hospital, ragama, SrLanka


Description A 29 year old patient presented with progressive dyspnea (mMRC grade 3) over 4 months. Her 2D Echocardiogram revealed moderate pulmonary hypertension with right ventricular dilatation. She had a history of inflammatory polyarthritis and constitutional symptoms. Her ANA was 1: 80 (Homogenous pattern). HRCT-Chest, CTPA, bubble contrast study and serology tests including APLS screening were all negative. A diagnosis of pulmonary arterial hypertension (PAH) was made. She also had an incidental detection of diffuse splenic calcification which is a rare association of SLE. She was treated with immunosuppressants and vasodilators for which she had a symptomatic improvement.

A 31 year old patient presented with progressive abdominal pain, distention and diarrhea for 2 weeks duration. She underwent an exploratory laparotomy which revealed gross ascites and dilated small intestinal loops. Subsequent Abdominal CT showed small intestinal wall ischemia involving multiple arterial territories. She also reported on alopecia and gave a history of ITP during her pregnancy in 2016. With positive ANA (1:1280 titer), low complement levels, negative APLS screening and exclusion of other causes, SLE with mesenteric vasculitis was confirmed.

A 40 year old patient presented with a 2 week history of fever, nonproductive cough, inflammatory type polyarthritis and bilateral axillary lymphadenopathy. She later developed a pancytopenia and alopecia. Her ANA was 1:10000; Diagnosis of SLE was made. Axillary lymph node biopsy showed necrotizing lymphadenopathy and immunohistochemistry was strongly positive for CD68 favoring Kikuchi disease.

Conclusions Initial clinical presentations of SLE can be highly variable; can mimic infections and malignancies and may lead to delays in diagnosis. Therefore the clinicians should be vigilant on rare presentations of SLE such as PAH, mesenteric vasculitis and necrotizing lymphadenopathy to initiate timely treatments. Timely management in mesenteric vasculitis is crucial to prevent the possible catastrophic complications like necrotic bowel, perforation and sepsis.

  • pulmonary arterial hypertension
  • mesenteric vasculitis
  • necrotizing lymphadenopathy

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