INTRODUCTION
Systemic sclerosis (SSc) is a systemic inflammatory rheumatic disease characterised by the presence of fibrosis of the skin and internal organs and obliterative vasculopathy in addition to immune system disorders. The disease usually occurs in women between the ages of 30 and 50, while it is a less common occurrence in men and the elderly. Studies have shown that clinical features differ between patients who develop the disease at a younger age compared to geriatric patients (1).
Elderly patients have an increased risk of developing pulmonary hypertension, heart and kidney failure, and pronounced muscle weakness and functional impairment. While comparing the laboratory findings of older and younger patients, high levels of alkaline phosphatase (ALP) and low levels of creatinine kinase (CK) were observed in older patients, as well as a high percentage of anti-centromere antibodies and low level of U1RNP antibodies, which were observed in immunological findings. On the other hand, elderly patients tend to have a mild form of Raynaud’s phenomenon as well as a lower risk of developing digital ulcers (2).
There is no doubt that high life expectancy in patients with SSc is associated with a poor prognosis, but it is important to note that most geriatric patients have other comorbidities which, in addition to the age factor, increase the risk of adverse outcomes and mortality.
In this paper, we shall present the case of an 84-year-old patient who came for a check-up due to pain and swelling of the small wrist joints with significantly reduced functional ability, and who was eventually diagnosed with SSc.
CASE REPORT
An eighty-four-year-old patient was referred for specialist treatment due to symptoms such as swelling and pain in his fingers and Raynaud’s phenomenon, which appeared several months ago. Various conditions such as arterial hypertension, coronary heart disease (after recovery from myocardial infarction) and scalp sarcoma surgery which occurred several years ago (without chemotherapy) were known from the patient’s medical history.
The clinical examination revealed abnormalities such as diffuse swelling of the fingers (“puffy fingers”), arthritis of the small wrist joints and both wrists, sclerodactyly, skin tightening of the upper extremities and chest, and all of these symptoms were enough to diagnose SSc based on the clinical features. Extremely high levels of inflammatory markers were observed in laboratory findings (ESR = 102 mm/hr, CRP = 81 g/L). Subsequent immunological findings included a positive antinuclear antibody finding with positive centromere-specific fluorescence (> 1: 640). The CK finding was in the normal range (21 U/L). Capillaroscopy revealed the presence of megacapillaries and pathological bleeding, which indicated an active scleroderma pattern. Pulmonary function tests showed reduced diffusion capacity for carbon monoxide (DLCO = 57%), and multi-slice computed tomography (MSCT) findings of the chest did not show a distinct interstitial pattern. The echocardiogram showed signs of ischemic cardiomyopathy with preserved ejection fraction, while no indirect signs of pulmonary hypertension were detected. The barium swallow test of the oesophagus showed no abnormalities, the chest MSCT showed no signs of oesophageal dilatation, the patient had no pronounced gastrointestinal disturbances, and did not agree to the recommended endoscopy. Tumour markers and abdominal magnetic resonance imaging showed no signs of associated active malignancy. Therapy was initiated by administering low doses of methylprednisolone (8 mg of methylprednisolone for two weeks, followed up with a dose of 4 mg for 5 months and ultimately a dose of 4 mg every other day) and peripheral vasodilators (amlodipine). There was an excellent clinical response to this therapy, in addition to the regression of peripheral arthritis and swelling of the fingers, as well as the regression of skin thickening. Normalisation of inflammatory parameters is observed in laboratory findings. At the follow-up appointments, the patient was in good general condition, his functional status was normal, and he was able to perform all normal daily activities. We continue to carry out the follow-up of this patient.
DISCUSSION
This paper presents the case of a patient who was diagnosed with SSc at the age of 84. Through this case report, it can be concluded that the clinical and laboratory characteristics of SSc, according to the data found in the reviewed literature, were different in the case of our geriatric patient compared to younger patients. SSc is a disease that most commonly occurs in the working age population, although its incidence has been reported in both younger and older patients. A relatively small number of cases in which patients were diagnosed with SSc late in life has been described in literature so far.
Hügle et al. have conducted a study which included 8554 patients with SSc. Out of the total number of patients, only 123 of them (1.4%) were older than 75, and the results showed that the elderly patients were more likely to have a limited type of SSc and a higher prevalence of anti-centromere antibodies. The modified Rodnan skin score and the prevalence of pulmonary fibrosis did not differ significantly between the two groups (3). According to the 2013 ACR/EULAR classification criteria, the skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc. In the case of our patient, it was determined at the first clinical examination that there were enough elements to raise suspicion of SSc. Other manifestations of the disease that collectively confirm the diagnosis of SSc (score ≥9) include: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries (detected through capillaroscopy), pulmonary arterial hypertension and/or interstitial lung disease, Raynaud’s phenomenon and SSc-related autoantibodies (4). Our patient had sclerodactyly and Raynaud’s phenomenon, a positive anti-centromere antibody finding (a type of anti-nuclear antibodies) was revealed through laboratory findings, while capillaroscopy revealed an active scleroderma pattern.
According to the published literature data, pulmonary hypertension diagnosed with an echocardiogram and right heart catheterisation are more common in patients with late onset of the disease, while the risk of digital ischemia is lower in these patients. The prevalence of anti-U1RNP antibodies is significantly lower in elderly patients (2). The ultrasound showed no signs of pulmonary hypertension in our patient, and according to the data found in the literature, the anti-U1RNP antibodies were negative. Despite the Raynaud’s phenomenon and an active scleroderma pattern (revealed through capillaroscopy), digital ulcerations were not present in our patient.
Gastrointestinal involvement is relatively high in patients with SSc. In a study conducted by Alba et al., patients with early onset of the disease have been shown to have a higher prevalence of oesophageal involvement (72% of early-onset patients compared to 56% of late-onset patients). After correction for the population effects of age and sex, mortality was shown to be higher in younger patients (1). The patient from this case report had no gastroenterological disorders or symptoms or signs typical of SSc, the barium swallow test of the oesophagus showed no abnormalities, and the chest MSCT showed no signs of oesophageal dilatation.
In our patient, high levels of alkaline phosphatase (ALP) and relatively low levels of creatinine kinase (CK) were recorded, in accordance with the data found in the literature (5).
Although it seemingly appeared like a typical clinical presentation of SSc, our patient’s case required additional caution and differential diagnosis, primarily due to the presence of extremely high inflammatory markers and pronounced Raynaud’s phenomenon, which could indicate an associated malignancy. It is important to note that Raynaud’s phenomenon could be one of the initial manifestations of an associated malignancy (6).
An earlier diagnosis of a malignancy that was cured several years ago is unlikely to be associated with the development of SSc, and our patient has not been treated with chemotherapy or other drugs that would be associated with the development of SSc.
It is interesting to note that in the treatment of our patient, a low dose of glucocorticoids led to a complete regression of clinical symptoms as well as an improvement in skin manifestations, i.e., it resulted in an almost complete remission of the disease.
Further reduction or an attempt of discontinuation of glucocorticoid therapy is planned, and in case of an adverse clinical response, other immunosuppressants, whose use is not limited in elderly patients, will be considered. However, the dose of these immunosuppressants should be adjusted in order to be in accordance with the renal and hepatic function, taking into account all comorbidities that may occur in elderly patients.
In contrast to the mild course of the disease and the good response to therapy we had in our patient, late-onset SSc is mainly associated with a more aggressive form of the disease (3).
The higher prevalence of extracutaneous organ involvement at the onset of the disease and the earlier occurrence of systemic manifestations in the elderly is the probable reason for the earlier initiation of treatment and diagnosis (1, 2, 7). Attention should also be paid to the group of younger patients with mild symptoms who were initially classified as cases of undifferentiated connective tissue disease and who tend to develop features of SSc later in life (8).
Although systemic sclerosis most often occurs in middle-aged people, its occurrence has also been reported in elderly patients. Clinical and laboratory characteristics as well as response to therapy are different in younger and older patients. There is no doubt that high life expectancy in patients with SSc is associated with a poor prognosis, but it is important to note that most geriatric patients have other comorbidities which, in addition to the age factor, increase the risk of adverse outcomes and mortality.
A relatively small number of cases with SSc onset in patients over the age of 80 has been described in literature so far. Our patient’s case report enables a better exchange of clinical experiences, facilitation in differential diagnosis and a diagnostic and therapeutic algorithm in patients with late-onset SSc. It is particularly interesting to note that the administration of low doses of glucocorticoids in our patient has produced satisfactory results in terms of cutaneous manifestations of the disease, in addition to having a beneficial effect on peripheral joints. In addition to being significant in terms of scientific contribution, this patient’s case report can also contribute to additional research into the pathophysiological mechanisms of the onset and progression of this disease in geriatric patients.
Conflict of interest statement: The authors declare no conflict of interest.