Features of interstitial lung damage in systemic scleroderma (literature review and authors’ own data)
L.P. Ananyeva, O.A. Koneva, L.V. Teplova, V.N. Lesnyak
1 V.A. Nasonova Research Institute of Rheumatology, Moscow
2 O.M. Filatov City Clinical Hospital, Moscow
3 Federal Scientific Clinical Center of Specialized Types of Medical Care and Medical Technologies, Moscow
Interstitial lung damage (ILD) in systemic scleroderma (progressive systemic sclerosis) adversely affects the prognosis and is one of the leading causes of death. Early diagnosis and timely treatment can improve the prognosis of the disease. Therefore, in patients with the Raynaud’s phenomenon, which is debut of the disease in most patients, it is necessary to pay attention to the signs characteristic of systemic rheumatic diseases (swelling of hands, myalgia in the proximal limbs, morning stiffness, arthralgia and arthritis, erythematous or hemorrhagic eruptions, xeronosus, dysphagia, anemia, cytopenia, etc.). According to the chest MSCT, ILD is detected in 80% of patients with SS. Histological findings are similar to that of idiopathic ILD, but the histological type does not determine the prognosis. The course of ILD in SS is relatively benign, often subclinical. According to the functional pulmonary tests, restrictive ventilation disorders are the most typical, and diffusing lung capacity is reduced in 80% of patients. Pulmonary tests have a prognostic value at the first examination – the lower the norm, the worse the prognosis. It is necessary to perform chest MSCT in all patients with a newly diagnosed SS, since the quantitative (prevalence) and qualitative (frosted glass, honeycomb lung) parameters influence the choice of therapeutic tactics. Treatment of ILD is carried out only in patients with obvious signs of progression determined by the dynamics of the decline in functional pulmonary function tests. For the treatment of ILD, immunosuppressants are used to slow down the progression. The drugs of choice include cyclophosphamide, an alternative to which is mycophenolate mofetil. Currently, genetically engineered biological preparations are being tested, in particular, tocilizumab, rituximab, and others.
About the Autors
Lidiya P. Ananyeva, MD, professor, head of the Laboratory of microcirculation and inflammation, V.A. Nasonova Research Institute of Rheumatology. Address: 115522, Moscow, 34a Kashyrskoe shosse. Tel.: +74996189092. E-mail: lpana@yandex.ru
Olga A. Koneva, PhD, senior researcher, Laboratory of microcirculation and inflammation, V.A. Nasonova Research Institute of Rheumatology. Address: 115522, Moscow, 34a Kashyrskoe shosse. Tel.: +74996159394. E-mail: alloy@yandex.ru
Lyudmila V. Teplova, PhD, head of 22nd Department of rheumatology, O.M. Filatov City Clinical Hospital. Address: 111539, Moscow, 23 Veshnyakovskaya St. Tel.: +74953751339. Е-mail: teplival@mail.ru
Viktor N. Lesnyak, PhD, head of the Department of radiology with the MRI rooms, Federal Scientific Clinical Center of Specialized Types of Medical Care and Medical Technologies. Address: 115682, Moscow, 28 Orekhovy bul.
Tel.: +74953956277. E-mail: lesnyak_kb83@mail.ru
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