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Depending on the extent of organism affected, there is a systemic (amyloid is deposited in the interstitial space of multiple tissues and organs) and localized (amyloid is deposited in one or a few solitary lesions) form of amyloidosis. Localized forms of amyloidosis have a significantly better prognosis than the systemic ones. The respiratory tract might be affected by diffuse interstitial involvement, associated with systemic AL-amyloidosis, as well as localised involvement of respiratory tract (localised laryngotracheobronchial amyloidosis) or pulmonary parenchyma called nodular form of localized pulmonary amyloidosis. Tracheobronchial form may affect larynx and bronchial tree, and forms plaques or nodules in the epithelium of the respiratory tract. Nodular form causes spherical or irregular lesions in the pulmonary parenchyma, indistinguishable from pulmonary parenchyma metastases. We describe a two-year follow up of a patient with nodular form of pulmonary amyloidosis. The patient had multiple lesions in both lungs, clearly visible on HRCT (High Resolution Computer Tomography) that intensively accumulated fluorodeoxyglucose (FDG) during the first PET-CT. At the time of diagnosis, the largest lesion SUV for FDG accumulation was 8.2. Histochemical analysis showed that amyloid consisted of the light λ chains, i.e. AL-amyloid. Investigations to detect a systemic form of amyloidosis, if present, were negative. The patient had no monoclonal immunoglobulin either in the urine or serum (negative immunofixation) and had normal levels of free light chains in the serum. Her symptoms were previously suggestive of the Sjögrens syndrome. However, the rheumatologist consulted at the time of diagnosis of the nodular form of pulmonary amyloidosis did not find any signs of an active systemic connective tissue disorder. CRP was repeatedly normal. When systemic AL-amyloidosis was excluded, we decided to only monitor lesion development with no treatment intervention. The patient had 3 PET-CTs. CT showed that no lesions enlarged, some lesions decreased in size slightly. It should be emphasized that follow-up PET-CTs did not show increased FDG accumulation. We assume that the increased FDG accumulation in pulmonary lesions seen during the first PET-CT was due to the activity of the cells that formed this amyloid and that this activity spontaneously ceased, leading to normalization of FDG accumulation in pulmonary nodules. PET-CT is useful for monitoring of the development of pulmonary nodular amyloidosis. Normalization of originally increased FDG accumulation in amyloid lesions suggests cessation of the process of amyloid formation and is a positive prognostic sign.

Castlemans disease (also called angiofollicular lymph node hyperplasia) can take two forms with different prognosis: the localized form can usually be treated by a surgical intervention and has therefore a favourable prognosis. On the other hand, the multicentric form has an unfavourable prognosis and requires systemic treatment. Classic manifestations of multicentric Castlemans disease are multiple sites of lymphadenopathy, sometimes hepatomegaly and also splenomegaly or serous cavity effusions. Typical pathological laboratory levels measured in patients with this disease include an increased CRP level, anaemia of chronic diseases, and many patients have an increased total protein concentration, in some cases exceeding even 100g/ l. It is caused by a high concentration of polyclonal immunoglobulins. Typical clinical symptoms include fluctuating subfebrile or febrile temperatures, increased night sweats and fatigue usually related to anaemia. In some patients, the disease is manifested as vasculitis, frequently also affecting cerebral arteries, i.e. leading to cerebrovascular accidents. The aetiology of this disease is unclear; it is a polyclonal lymphocyte proliferation, often with differentiation into plasma cells. It is not a clonal malign disease; however, it can transform into a clonal lymphoproliferative disease. Even though it is not a malign disease in the histomorphological sense, the disease symptoms are so acute that systemic treatment is required. In the past, the treatment method of this disease used to be based on corticoids and cytostatics; however, such treatment was not always successful in achieving its objective, i.e. complete remission. In the past few years, an improvement of treatment results was accomplished by adding a new drug to the basic medication, i.e. to cytostatics and dexamethasone. Many publications describe the benefi t of adding a third drug from the IMiDs group (immunomodulatory drugs), such as thalidomide or lenalidomide. These drugs affect the formation of cytokines and block the angiogenesis, which in turn positively influences the speed of the treatment response. The second new drug that has helped in combination with classical treatment is the anti-CD20 antibody, rituximab. The third new drug to add this list is the monoclonal antibody against the interleukin-6 receptor, tocilizumab. This paper describes a rapid treatment response after combined treatment with cyclophosphamide 500mg/ m2 i.v. infusion 1st and 15th day in a 28- day cycle, dexamethasone 20mg p.o. cycle day 1- 4 and cycle day 15- 18, and thalidomide 100mg daily. In the course of the two-month treatment, the accumulation of fl uorodeoxyglucose during the PET-CT imaging has normalized; the originally pathologically enlarged nodes have become smaller, the originally elevated CRP level has normalized and the originally signifi cantly lower haemoglobin level has risen. This is the second patient with multicentric Castlemans disease in the last three years who showed a rapid response to treatment with thalidomide combined with cyclophosphamide and dexamethasone. Therefore, we consider such treatment suitable for newly diagnosed patients with multicentric Castlemans disease.

We describe a case of an Erdheim-Chester disease patient. First line chemotherapy treatment with 2-chlorodeoxyadenosine did not reduce fluorodeoxyglucose accumulation in pathological lesions. The patient had continuously increased CRP values of 17-20 mg/l. The disease continued to cause subfebrile temperatures and significant fatigue that made the patient to spend most of the daytime in bed. To manage the permanently increased inflammation markers, we decided to start treatment with anakinra, successfully used in some other autoinflammatory diseases (e.g. Schnitzler syndrome). We have now been able to evaluate the first 6 months of treatment. Daily subcutaneous administration of anakinra (KineretTM 100 mg daily) led to normalization of CRP values, cessation of subfebrile temperatures and, importantly, significant reduction of fatigue. Time periods the patient was able to spend out of the bed increased significantly. Consequent to the reduced fatigue, the patient was able to perform basic household tasks he was unable to undertake without treatment. After 3 months of treatment, fatigue of the same intensity returned following a short interruption of therapy. The CRP values went up again to 12 mg/l. CRP value returned back to norm and fatigue ceased after re-initiation of daily Kineret injections. Objective treatment response was assessed by measuring the degree of fluorodeoxyglucose accumulation in pathological bone lesions. PET-CT was performed before and 3 and 6 months after anakinra initiation. Intensity of accumulation did not change significantly after the first 3 months of therapy but decreased after 6 month therapy. Follow up CT of abdominal cavity was performed at the end of the 6th month of treatment. Presented CT images from before and 6 months after the treatment evidence an obvious reduction in fibroid changes in the retroperitoneum. Daily administration of anakinra to a patient with active Erdheim-Chester disease significantly reduced intensity of fatigue and improved quality of life, led to a reduction in inflammatory markers and regression in retroperitoneal fibrotization.