Abstract | November 18, 2023

Anasarca as First Sign of Late-Onset Lupus

Renato Ferrandiz Espadin, MD, Internal medicine resident PGY2, North Alabama Medical, Florence, AL

Sarah Elsayed, MD, Internal Medicine, PGY2, North Alabama Medical Center, Eugene Jalbert, DO, Rheumatologist, North Alabama Medical Center

Learning Objectives

  1. SLE commonly affects women of childbearing age. However, 3–18% of people over 50, predominantly men develop late-onset Lupus.
  2. In late-onset Lupus, serositis, lung involvement, and associated Sjogren's syndrome are more common than cutaneous symptoms, photosensitivity, arthritis, and nephritis. There are higher titers for rheumatoid factors, anti-Ro and anti-La antibodies; and lower anti-RNP and anti-D antibodies.
  3. These patients are three times more likely to develop ILD. It is unclear if it is caused by the disease, overlap between SLE and Sjogren's Syndrome (SLE-SS), aging, or smoking.
  4. Late-onset lupus patients have more severe chronic kidney lesions of glomerular sclerosis, interstitial fibrosis, tubular atrophy, and cortical wall thickening than early-onset Lupus patients, even if the active lesions were equal. It is unknown if the renal abnormalities result from SLE activity alone or also the result of aging.
  5. Late-onset lupus has a lower survival rate and higher risk of death compared to early-onset SLE. It is unclear if this is due to a more aggressive disease course, the presence of other co-morbidities at the time of diagnosis, or a combination of these variables.

An 84-year-old man presented with exertional dyspnea, orthopnea, productive cough with clear sputum, left-side pleuritic chest pain, oliguria, and anasarca for one week. He was a former smoker with history of hypertension, untreated interstitial lung disease (ILD) and chronic back pain. Vitals were remarkable for BP of 168/86mmHg, tachypnea at 24 breath/min and hypoxemia with SPo2 of 95% on 3 L/min oxygen. On examination, he was afebrile, had decreased breath sounds bilaterally; firm, diffusely tender, distended abdomen; +2 bilateral upper and lower limbs edema; otherwise, normal.

Labs revealed mild microcytic anemia and deranged renal functions. BNP was elevated at 2840 Pg/ml. Urine studies showed nephrotic range proteinuria. EKG sinus rhythm 71 bpm with left atrial enlargement. CXR showed Chronic bilateral interstitial infiltrates and mild cardiomegaly, unchanged from the previous. Interstitial lung disease was evident on the CT chest showing bilateral lung fibrotic changes with honeycombing. Transthoracic echo showed normal left ventricular size, diastolic and systolic function with EF 55–60%. Serology excluded influenza, COVID-19, HIV, and viral hepatitis.

Further tests revealed positive ANA by IgG and IFA 1:640 (Speckled), SSa by Ro52 (1680) and Ro60 (>1378) and SSb (19), rheumatoid factor by IgA (27), C1q (29.7), PT by IgM (46), normal C3/C4, PC4d, and negative ANCA, suggesting a diagnosis of Sjogren and Systemic Lupus. Renal biopsy showed class V membrane lupus nephritis. The patient received prednisone, hydroxychloroquine, and mycophenolate with dramatic improvement of his symptoms and renal functions and was discharged with rheumatology follow-up.

References and Resources

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  2. Rovenský J, Tuchynová A. Systemic lupus erythematosus in the elderly. Autoimmun Rev. 2008;7(3):235-239. doi:10.1016/j.autrev.2007.11.014
  3. Tian N, Zhou Q, Yin P, et al. Long-Term Kidney Prognosis and Pathological Characteristics of Late-Onset Lupus Nephritis [published correction appears in Front Med (Lausanne). 2022 Sep 05;9:1018311]. Front Med (Lausanne). 2022;9:882692. Published 2022 May 30. doi:10.3389/fmed.2022.882692
  4. Riveros Frutos A, Holgado S, Sanvisens Bergé A, et al. Late-onset versus early-onset systemic lupus: characteristics and outcome in a national multicentre register (RELESSER). Rheumatology (Oxford). 2021;60(4):1793-1803. doi:10.1093/rheumatology/keaa477
  5. Medlin JL, Hansen KE, McCoy SS, Bartels CM. Pulmonary manifestations in late versus early systemic lupus erythematosus: A systematic review and meta-analysis. Semin Arthritis Rheum. 2018;48(2):198-204. doi:10.1016/j.semarthrit.2018.01.010
  6. Maddison PJ. Systemic lupus erythematosus in the elderly. J Rheumatol Suppl. 1987;14 Suppl 13:182-187.
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