How Does Age at Onset Influence the Outcome of Autoimmune Diseases?
Table 1
Clinical differences between early and adult onset.
AD
Early onset
Late onset
SLE
Higher degree of morbidity [12] Higher (SLEDAI) score at presentation Higher incidence of renal disease Malar rashes Pericarditis Hepatosplenomegaly Hematologic alterations (Leucopenia) Higher use of prednisone Additional immunosuppressive therapies Greater lifetime of damages from the disease flares and the treatment side effects [13] 2 times higher mortality rate Growth failure Delayed puberty Fibromyalgia [11] Higher odds of presenting proteinuria Haemolytic anaemia Arthritis [16]
Increased rate of pulmonary disease [11] Increased rate of simultaneously developing another AD [12]
RA
Proximal Interphalangeal, metacarpophalangeal, elbow, metatarsophalangeal, and ankle joints Classical rheumatoid hand deformities Interstitial lung disease Associated SS [18] Shorter morning stiffness [19]
Acute onset in large and small joints (specially shoulders) PMR-like symptoms [20] Constitutional features Weight loss Myalgia Rheumatic nodules Neuropathy [18] Longer morning stiffness [19]
Positive RF PJIA* Rapid onset of inflammation in multiple joints Proximal interphalangeal, metacarpophalangeal, elbow, metatarsophalangeal, and ankle joints Effects of the disease in a growing skeleton: Growth retardation Accelerated growth of an affected joint [21]
Ketosis and ketoacidosis Higher mean glycated hemoglobin [24, 25]
Better preserved B-cell function Longer symptomatic period before diagnosis Less frequency of insulin autoantibodies and HLA class II susceptibility alleles [25, 26] Milder signs of metabolic decompensation and a lower glycated hemoglobin level at diagnosis [27, 28]
AD: autoimmune disease; RA: rheumatoid arthritis; SS: Sjögren’s syndrome; T1D: type 1 diabetes; MS: multiple sclerosis; AITD: autoimmune thyroiditis; PJIA: polyarticular juvenile idiopathic arthritis; RF: rheumatoid factor.
*In RA, early age at onset is considered ≥16 years old. Positive RF PJIA is considered a comparable disease with a childhood onset (<16 years old).