Difficulties in systemic sclerosis diagnosis on the example of clinical case
Introduction. Systemic sclerosis (SSc) is a rare chronic systemic disease of the connective tissue, presenting with high level of patient-to-patient clinical variability of features. Objectives. The aim of our research was to study a clinical case of the clinical course and difficulties in timely diagnosis of SSc in middle-age female patient. Materials and methods. Patient is a 39-year-old woman unemployed due to disability of 3rd degree. The disease manifested with Raynaud phenomenon in 2008. During 2008-2013 condition of the patient progressively worsened, new symptoms appeared as difficulty in swallowing of solid food, ulcers. In 2013 she was consulted by a surgeon and treated with antibiotics without significant effect. In October 2013 she was admitted to the rheumatology department, where she was eventually diagnosed with SSc, stage II (generalized), subacute course, activity II stage, with damage to the skin (edema, induration), blood vessels (Raynaud syndrome, stage III ischemia), joints (polyarthritis with predominant lesions of the hands, wrists, elbow, knee, feet joints, radiographic changes of II grade, functional impairment I st.), esophagus (esophagitis). The last hospitalization was due to the worsening of the general condition. Patient was treated with methylprednisolone 8 mg, amlodipine 2,5 mg, naftidrofuryl 200 mg tid, aspirin 75 mg, pentoxifylline 600 mg, omeprazole 20 mg. Results. Our study presents the results of different laboratory and instrumental tests conducted in the clinic: full blood count, biochemical panel, serological tests (antibodies), ECG, EchoCG, chest X-ray, upper Gl tract radiography, ultrasound of abdominal organs, kidneys, rheovasography of the vessels of the upper limbs, X-ray of hands. Patient was consulted by gastroenterologist, vascular surgeon and gynecologist. Conclusions. An important task for the practicing physician is the ability to recognize the first signs of systemic sclerosis - “red flags”, which will help to diagnose the disease at early stages, start timely treatment to prevent irreversible pathological changes, early disability and mortality of patients with SSc.
Bilovol, O. M., Kniazkova, I. I. Cystemna sklerodermiia: klinika, diahnostyka, likuvannia [electronic resource] // Vnutrenniaia medytsyna. 2008. 1(7). Access to resourse: http://www.mif-ua.com/archive/ article/4383. [in Ukrainian].
Schwartz, R. A., Zalewska, A., Systemic sclerosis [electronic resource] // Medscape. – 2019. – Access to resourse: https://emedicine.medscape.com/article/1066280-overview#showall.
Shostak, N. A., Klimenko, A. A. Sistemnaya sklerodermiya: sovremennaya klassifikatsiya i metodyi lecheniya // Lechebnoe delo. 2009. 4. P. 4–11. [in Russian].
Jimenez, S. A., Mendoza, F. A. Scleroderma [electronic resource] // Medscape. 2018. Access to resourse: https://emedicine.medscape.com/article/331864-overview#showall.
Starovoytova, M. N., Porazhenie kozhi pri sistemnoy sklerodermii // Nauchno-prakticheskaya revmatologiya. 2010. 2. P. 83–-86. [in Russian].
Van den Hoogen, F., Khanna, D. Fransen, J. [et al.] 2013 classification criteria for systemic sclerosis: an American college of rheumatology / European league against rheumatism collaborative initiative. // Ann Rheum Dis. 2013. 72. – P. 1747–1755.
Klinichnyi protokol nadannia medychnoi dopomohy khvorym iz systemnoiu sklerodermiieiu (SSD) // Ukrainskyi revmatolohichnyi zhurnal. 2007. 1 (27). P. 80. (in Ukrainian)
Distler, O., Allanore, Y., Denton, C. P. [et al.] Factors influencing early referral, early diagnosis and management in patients with diffuse cutaneous systemic sclerosis // Rheumatology. 2018. 57. P. 813–817.
Bellando, Randone, S., Guiducci, S., Matucci-Cerinic, M. Very early diagnosis of systemic sclerosis // Pol Arch Med Wewn. 2012. 122 (Suppl 1.). P. 18–23.
Vrancken A., De Langhe E., Westhovens R. [et al.] Difficulties in diagnosis of systemic sclerosis-related interstitial lung disease // Respirology Case Reports. 2015. 3 (3). P. 99–101.
Lepri, G., Bellando, Randone, S., Bruni C. [et al.] Very early and early diagnosis of systemic sclerosis [electronic resource] // Future Medicine. 2013. Access to resourse: https://www.futuremedicine.com/doi/full/10.2217/ebo.13.169.
Bellando Randone, S., Guiducci, S., Matucci-Cerinic, M. Patient subgroups and potential risk factors in systemic sclerosis: is there a possibility of an early diagnosis? // Int. J. Clin. Rheumatol. 2010. 5 (5). P. 555–564.
Meyer, O. C., Fertig, N., Lucas, M. [et al.] Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis. // The Journal of Rheumatology. 2007. Vol.34 (1). P. 104–109.
Hudson, M., Thombs, B., Baron, M. Time to Diagnosis in Systemic Sclerosis: Is Sex a Factor? // Pol Arthritis & Rheumatism (Arthritis Care & Research). 2009. Vol.61 (2). – P. 274–278.
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