Thoracic Manifestation of Granulomatosis with Polyangiitis: A Case Report.

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated disorder with necrotic vasculitis of small- and medium-size arteries and veins. In the literature, there are many case reports of patients with GPA of different, sometimes unusual, clinical manifestations. In this paper, we present difficulties that accompanied the process of diagnosing GPA in a 54-year-old symptomatic patient who was. Computer tomography scans showed numerous tumor-like lesions of various and irregular sizes in both lungs. Positron emission tomography scans suggested a lymphoproliferative disease, otherwise failing to provide a clue concerning its nature or localization. After a series of diagnostic twists and turns, inclusive of bronchoalveolar lavage, cervical mediastinoscopy, paratracheal lymph biopsy, and histopathologic examinations, and other tests, the diagnosis of GPA was established as the most probable. The patient was acutely treated with loading doses of methylprednisolone and cyclophosphamide, gradually tapered off during the long-term follow-up. He was discharged from the hospital in a good condition. We conclude that GPA is an uncommon disease with indistinctive signs, which raises the risk of its being overlooked. A diagnostic algorithm is required for patients with suspected GPA. A timely diagnosis is essential as the disease may quickly progress into renal or multiorgan dysfunction, and ultimately lead to death if untreated. Pulmonary involvement may also suggest neoplastic changes.

Pathophysiology of hyperuricemia and its clinical significance - a narrative review.

Hyperuricemia, i.e. increased serum uric acid (UA) concentration, is a common problem in clinical practice. While there are clear guidelines concerning management of symptomatic hyperuricemia in acute conditions such as gout, urolithiasis or acute urate nephropathy, less is known about their secondary prevention. Moreover, despite the ongoing debate on the role of UA in the pathogenesis of chronic kidney disease, hypertension, cardiovascular disease and heart failure, the management of asymptomatic hyperuricemia in patients with these chronic conditions is still mainly up to physicians' judgement. Individual considerations should always be taken into account when prescribing urate-lowering therapy. In this narrative review study, we attempt to present current trends concerning treatment of patients with either symptomatic or asymptomatic hyperuricemia in the light of the available knowledge on the role of hyperuricemia in the development of gout, renal, cardiovascular and other diseases.

Tofacitinib in the treatment of active rheumatoid arthritis - single-centre experience.

OBJECTIVES: To assess the efficacy and safety profile of tofacitinib taken orally at a dose of 10 mg/day in patients with severe active rheumatoid arthritis (RA). MATERIAL AND METHODS: The retrospective observational study included 10 patients (6 women and 4 men) with RA treated with tofacitinib. All the patients had high disease activity (DAS28 [ESR] > 5.1), despite therapy with two synthetic disease-modifying antirheumatic drugs (DMARDs). Before the initiation of treatment, routine laboratory tests were performed, and disease activity was assessed in all the subjects. RESULTS: The mean age of the patients in the study group was 58.18 years (43-67). The average duration of RA was 9.9 years (2-24). The mean baseline value of DAS28 (ESR) was 6.37. Tofacitinib was used in combination with a conventional DMARD in all study subjects: with methotrexate in the majority of patients, and with leflunomide and an antimalarial drug in three patients. The mean duration of therapy with tofacitinib was 7.57 months (3.9-10.8). A significant decrease in the disease activity was observed (p < 0.05). A reduction in DAS28 (ESR) score was seen already after the first month of therapy, and the trend was maintained during subsequent months of follow-up. The mean value of DAS28 (ESR) after 6 months was 2.78. A slight increase in the serum concentration of HDL cholesterol was observed during treatment. In one patient symptoms of chronic upper respiratory tract infection led to discontinuation of the drug. The observed adverse events were of mild/moderate degree. CONCLUSIONS: The results of our retrospective observational study conducted in the setting of daily clinical practice confirm a good clinical response to tofacitinib. Despite the observed adverse effects, in the light of the available data tofacitinib demonstrates a favourable safety profile. JAK kinase inhibitors - a new class of drugs - will enable a wider population of patients to achieve remission or low disease activity.

Relapsing polychondritis - analysis of symptoms and criteria.

OBJECTIVES: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context of data from available published studies. MATERIAL AND METHODS: A retrospective case analysis of 10 patients with symptoms of RP, hospitalised at the Department of Rheumatology and Internal Diseases of Wroclaw University Hospital between January 2008 and December 2018. RESULTS: Nine out of 10 patients fulfilled at least one of the three sets of the diagnostic criteria. The mean age (±standard deviation) at diagnosis was 54.4 ±13.3 years and ranged from 32 to 73 years. The symptoms suggestive of the RP diagnosis were mainly inflammation of the pinna (in 80% of patients) and laryngeal stenosis (in 20% of patients). The mean age at which initial symptoms were observed was 52.3 ±12.0 years and ranged from 31 to 69 years. Auricular chondritis was the first manifestation of the disease in 40% of cases (two women and two men) laryngeal chondritis in 20%, nasal chondritis in 10%, and bronchial stenosis in 10%. Other initial symptoms were polyarthritis, which was present in 10% of cases (male) and general symptoms observed in 10%. CONCLUSIONS: A thorough analysis of the entire medical history with specific questions about the occurrence of the manifestations of the disease in the past leads to the diagnosis of RP. The RP also should be considered in differential diagnosis of respiratory track narrowings. It is very useful to apply the three sets of criteria simultaneously in the diagnostic process.