Vitamin D supply in patients with rheumatic diseases in Poland - a pilot study.

OBJECTIVES: In rheumatic diseases, vitamin D supply is recommended as part of the prophylaxis and treatment of osteoporosis, especially in patients undergoing glucocorticoid therapy, but also due to its immunoregulatory and anti-inflammatory properties. We aimed to evaluate serum 25-hydroxyvitamin D [25(OH)D3] levels in Polish patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA), in relation to various clinical parameters, and to assess the initial range of doses for the purpose of further research. MATERIAL AND METHODS: 112 patients (39 with SLE, 44 with SSc and 29 with GPA), referred to the Department of Rheumatology and Internal Medicine in Poznan, Poland, were enrolled in this retrospective study. Demographic and clinical data were collected, including 25(OH)D3 serum levels, vitamin D supplementation doses and season of blood sampling. RESULTS: Mean (SD) serum 25(OH)D3 concentrations were 31 (19.4) ng/ml for SLE, 28.8 (12.5) ng/ml for SSc and 28 (15.2) ng/ml for GPA, and they did not significantly differ between the groups. Vitamin D levels below the optimal range were found in 43.8% of SLE, 65.9% of SSc and 72.4% of GPA patients. 80% of patients reported vitamin D intake, with a mean daily dose of 1398 IU for SLE, 1345 IU for SSc and 1689 IU for GPA. Vitamin D insufficiency and deficiency were frequent among patients with rheumatic diseases, independently of the diagnosis and season. CONCLUSIONS: Patients with rheumatic diseases seem to require higher doses of vitamin D than recommended for the general population. The present results indicate the necessity to use higher initial doses of vitamin D in this group of patients (2000 to 4000 UI) and to maintain the dose of vitamin D regardless of the change of seasons.

Systemic Sclerosis and Serum Content of Transforming Growth Factor.

Systemic sclerosis is a connective tissue disease characterized by tissue fibrosis leading to interstitial lung disease. Transforming growth factor-ß (TGF-ß) has been of interest as a potential diagnostic marker and also as a drug target in systemic sclerosis. The aim of this study was to assess the serum content of TGF-ß1 in patients with systemic sclerosis and to assess its potential role in tissue fibrosis. The study included 30 patients, 5 men and 25 women, of the mean age of 46.9 ± 12.8 years, diagnosed with systemic sclerosis. The control group consisted of 19 women of the mean age of 28.4 ± 7.8 years, diagnosed with primary Raynaud's disease. TGF-ß1 serum levels were measured, chest imaging examinations were performed, and fibrotic tissue changes were assessed using the modified Rodnan Skin Score. We found that the mean serum TGF-ß1 content in patients with systemic sclerosis was 598.7 ± 242.6 pg/mL, whereas it was 568.4 ± 322.2 pg/mL in the control group (p = 0.378). We also failed to substantiate any significant relationship between TGF-ß1 serum levels and the severity of pulmonary and skin fibrosis in systemic sclerosis. In conclusion, systemic sclerosis does not seem a disease that would be accompanied by a specific enhancement of serum TGF-ß1. Thus, this cytokine is rather unlikely to play an essential role in the development and course of the disease, nor can it be considered diagnostic or prognostic marker.

Chikungunya virus: a rheumatologist's perspective.

Chikungunya virus (CHIKV) is an arthropod-borne alphavirus, transmitted by Aedes aegypti and Aedes albopictus mosquitoes. It is responsible for a febrile illness, typically accompanied by maculopapular rash and severe, incapacitating arthralgia. The disease, although generally self-limiting, frequently evolves into a long-lasting, debilitating rheumatic disorder, which shares many clinical features with rheumatoid arthritis (RA). The underlying mechanism by which CHIKV induces persistent arthritis remains under investigation, however, currently, attention is drawn to the fact, that chronic chikungunya (CHIK) and RA have many common cellular and cytokine pathways involved in their pathogenesis. Over the past decades, the virus has dispersed unexpectedly from tropical and subtropical regions of Africa and Asia, affecting millions of people worldwide. No licensed vaccine, nor antiviral drug against CHIKV is yet available. Treatment of acute CHIK is symptomatic, whereas in chronic stages, different disease-modifying anti-rheumatic drugs (DMARDs) have been used with variable success. Hence, chronic CHIK is an emerging rheumatic condition that rheumatologists have to deal with. This review provides brief insights into the epidemiology, pathogenesis, clinical features and management of Chikungunya disease, with special regard to post-chikungunya rheumatic disorder and its relationship with RA.

Retroperitoneal fibrosis - a report of five cases.

Retroperitoneal fibrosis (RPF) is a rare disease, characterized by inflammation and deposition of fibrotic tissue in the vicinity of the abdominal aorta and iliac arteries. We present a report of five patients admitted to our department between January 2014 and February 2017, diagnosed with RPF. Abdominal pain was the most common presenting symptom; however, in one patient, RPF was identified accidentally in routinely performed ultrasonography. In 4 cases, corticosteroids (CS) in combination with azathioprine were applied as first-line therapy, whereas one patient was treated with intravenous methylprednisolone pulses followed by oral CS. In this paper, clinical features as well as laboratory and radiographic findings together with management and treatment outcomes in patients with RPF are discussed. Given the rarity of the condition, it seems important to report every single case of RPF to help establish its management algorithm.

Retroperitoneal fibrosis - the state-of-the-art.

Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed.