Late-Onset Sarcoidosis in a 77-Year-Old Man
To the Editor: Sarcoidosis is rare in elderly adults;1 we report here a case in an elderly adult hospitalized for confusion.
A 77-year-old man, who had been independent until then, was hospitalized for deteriorated general health and confusion. His history included ischemic cardiopathy, hiatal hernia, and hypovitaminosis D. Over the previous 8 days, he had become totally dependent and confused, with inappropriate undressing and urination. His treatment consisted of nebivolol, clopidogrel, pravadual, nicorandil, omeprazole, and cholecalciferol. Clinical examination revealed asthenia, anorexia, and arterial hypertension (185/105 mmHg). Biological tests showed mild normochromic normocytic anemia (12.5 g/dL; normal 13–18), lymphocytopenia (900 cells/μL; normal: 1,500–4,000 cells/μL), slight thrombocytopenia (145,000/μL, normal 150,000–450,000/μL), hypoalbuminemia (31.6 g/L, (normal 35–40 g/L), hypercreatininemia (270 μmol/L, normal 59–104 μmol/L), hyperuremia (20 mmol/L, normal 2.1–7.1 mmol/L), hypercalcemia corrected at 3.58 mmol/L (normal 2.10–2.55 mmol/L), and intact hypoparathyroidism (3.8 ng/L, normal 15–65 ng/L). The electrocardiogram showed second-degree atrioventricular block. Chest radiography showed no cardiopulmonary anomalies. Bone scintigraphy and radiography of the ribcage and dorsolumbosacral spine revealed uncomplicated lumbosacral discarthrosis. Protein electrophoresis was normal. Angiotensin-converting enzyme (ACE) levels were high (82 U of ACE activity; normal <68 U of ACE activity). Cerebral magnetic resonance imaging (MRI) performed because of his confusion showed hypersignals in the bilateral paraventricular white matter, cortico-subcortical atrophy, and leukoencephalopathy. However, thoracic computed tomography revealed several incompressible mediastinal lymphadenopathies, with no infiltration in the lung parenchyma. Positron emission tomography with F-fluorodeoxyglucose showed major hypermetabolic fixation in the mediastinum and to a lesser degree in the myocardium, skin, and thorax. Pathological examination of lymph node biopsy showed epithelioid lymphadenitis and giant follicular lymphadenopathy, confirming sarcoidosis (Figure 1).
Management consisted of hyperhydration, infusions of bisphosphonates, and a low-calcium diet, leading to regression of the hypercalcemia. Oral corticotherapy at 1 mg/kg per day of prednisone equivalent was initiated to treat the sarcoidosis. The clinical and biological evolution was progressively favorable. The National Reference Centre for Sarcoidosis was asked for an opinion, because the possibility of cerebral involvement could not be excluded given the hypersignals.
Two and a half years later, the man was in good general health and was once again independent, with no behavior disorders. Calcemia and ACE levels were normal. Corticotherapy was reduced to 5 mg/d of prednisone equivalent.
Discussion
Even though sarcoidosis can be seen at any age, its incidence is highest in people aged 25 to 45 (70% of cases).1, 2 In northern Europe, a second peak of incidence is seen in perimenopausal women.1 Late-onset sarcoidosis, diagnosed in those aged 65 and older is rare, and the female/male ratio of 1.2 to 1.5 is even greater in elderly adults, reaching 5.1,1, 3 which makes this case unusual. Sarcoidosis seems to be linked to various factors, including genetic predisposition and specific environmental factors.1, 2 Clinically, there is intrathoracic involvement in 85% to 95% of cases, associated with extrapulmonary location (heart, nervous system, skin, eyes, joints) in half of cases, whereas extrapulmonary location without lung involvement may be seen in 5% to 15% of cases.4 Sarcoidosis is characterized by the heterogeneous nature of the clinical signs and evolution. In elderly adults, general signs dominate the clinical picture, with deteriorated general health, pulmonary involvement, and skin signs other than erythema nodosum, which like radiological signs, is not a classical sign in elderly adults.3, 5, 6 In the current case report, sarcoidosis was evoked because of the association of the deteriorated general health, confusion, hypercalcemia, cardiac conduction disorders, and high ACE level. Four cases of neurological involvement are mentioned in published series of late-onset sarcoidosis, including two cases of peripheral neuropathy, one of central neurological involvement, and one of myopathy.3, 5, 6 In this report, cerebral MRI showed no brain lesions characteristic of sarcoidosis, such as pseudotumors or meningeal thickening.1 Therefore, central nervous system involvement could not be confirmed, although the favorable evolution of cognitive function in the medium and long term with corticotherapy could suggest that the central nervous system was involved. The implication of hypercalcemia alone in the onset of confusion must also be considered.
Treatment of sarcoidosis in elderly adults is no different from that in younger individuals and is based on first-line oral corticotherapy.1-3, 6 The prescriber must bear in mind comorbidities, which are often more numerous and more severe in elderly adults, and the risk of adverse effects.7
Sarcoidosis in elderly adults is probably underdiagnosed. A thorough clinical examination and biopsy are essential to avoid missing this diagnosis.
Acknowledgments
The authors are grateful to Mr. Philip Bastable.
Conflict of Interest: None.
Author Contributions: Aline Ramanantsoa: study concept, acquisition of subject and data, interpretation of data, preparation of manuscript. Mamy Ramanantsoa: acquisition of subject and data, interpretation of data, preparation of manuscript. Elodie Bertrand: interpretation of data, preparation of manuscript. Dominique Valeyre, Christian Spiekermann: interpretation of data. Henri Patouraux: acquisition of subject and data, interpretation of data. Patrick Manckoundia: study concept, interpretation of data, preparation of manuscript.
Sponsor's Role: There was no sponsor for this study.
