Pleural amyloidosis as the first sign of IgD multiple myeloma
(胸膜淀粉样病变是IgD型多发性骨髓瘤的第一症状)

Clinical Rheumatology

Christian Hubert Roux¹ , V. Breuil¹, O. Brocq¹ and L. Euller-Ziegler¹

Received: 16 April 2004  Accepted: 23 August 2004  Published online: 1 December 2004

Abstract  We describe a case of IgD myeloma with amyloid and plasmocytic pleural localisations. At the onset of the disease it mimicked rheumatoid arthritis, which can be the first presentation of both AL amyloidosis and multiple myeloma. Pleural effusion can happen first in IgD myeloma, but our observation is of interest in that it confirms the very rare possibility of pleural amyloid and plasmocytic localisations devoid of pleural effusion.

Keywords  Amyloidosis - Multiple myeloma - Plasmocytic pleural effusion

Respiratory manifestations of amyloidosis are normally bronchotracheal or diffuse parenchymatous. Pleural localisations seem to be very rare [1–3] with mainly pleural effusions. Amyloidosis occurs at a frequency of 15% in cases of multiple myeloma (MM). Joint involvement is rare and accounts for less than 5% of cases [4], with a clinical spectrum ranging from indolent arthritis to rapidly destructive and painful inflammatory arthritis.

In the 11 cases recently reported [5], the most frequent manifestation was bilateral shoulder arthritis, followed by carpal tunnel syndrome and wrist localisations. Elbows, hips, knees, ankles and neck sites and polyarticular involvement mimicking rheumatoid arthritis (RA) were less frequent.

Case report

A 48-year-old woman who had been suffering from polyarthralgia for 4 years presented with an increase in inflammatory pain at several levels: hands, metacarpophalangeal joints, shoulders and knees. Biological inflammatory abnormalities were detected: erythrocyte sedimentation rate 87/125, C-reactive protein (CRP) 5 mg/l, rheumatoid factor negative (20 kUI/l), anaemia (haemoglobin 8 g/dl) and moderate hypercalcemia (2.58 mmol/l). Both kidney function and protein levels were normal. Standard chest X-ray showed a right suprahilar round image 25 mm in diameter (Fig. 1a). Computed tomography (CT) scan showed a pleural mass (Fig. 1b). Three biopsy samples of the mass were studied. They showed atypical plasmocytic nodes with large cells whose nuclei were eccentric. They also showed numerous large nucleoli with Congo red-positive amyloid deposits.

Fig. 1  a Standard chest X-ray showed a right suprahilar round image 25 mm in diameter. b CT scan showed a pleural mass

Serum protein immunoelectrophoresis detected IgD lambda monoclonal dysglobulinaemia; the IgA level was decreased (0.13 g/l) as well as IgG (5.68 g/l) and IgM (0.11 g/l). Bence Jones proteinuria was detected (3.23 g/l). Bone marrow aspiration and biopsy showed atypical plasmocyte proliferation. Standard X-rays of the skull showed typical erosions. Magnetic resonance imaging (MRI) detected myelomatous lesions at T1 and T2 and pelvic levels. Digestive biopsies previously performed because of anaemia were re-examined and ileal amyloidosis was discovered.

Four cycles of chemotherapy (vincristine, adriamycin and dexamethasone) and two autografts of peripheral stem cells were very effective and induced a 2-year-long complete remission before a relapse.

Discussion

The first manifestation in this case of IgD myeloma was pleural amyloidosis with a striking discovery by X-ray of a pleural myelomatous extramedullary localisation devoid of pleural effusion, which is usually found.

Extramedullary manifestations are common with IgD myelomas. Hyperproteinemia is rare and lambda light chain type is the most frequent (up to 89%). These light chains could be the result of amyloidosis and kidney insufficiency. Bone pains are the most frequent manifestations of IgD myeloma (72%), fatigue in 26% of cases only, weight loss in 19% and amyloidosis in 19%.

What is interesting here is that at the onset of the disease it mimicked RA, which can be the first symptom of both AL amyloidosis and MM [6]. Peripheral amyloid arthropathy is unusual (3%); the spectrum of possible rheumatological manifestations varies from indolent arthritis to painful and rapidly destructive inflammatory arthritis. Some are suggestive, such as hypertrophic shoulder arthropathy. Others are more misleading, especially polyarticular involvement; it may mimic RA when amyloid arthropathies are symmetrical and involve hands [7] or polymyalgia rheumatica when amyloid arthropathy predominates in shoulders and hips. Pleural effusion can happen first in IgD myeloma [8]. Our observation is of interest in that it confirms the very rare possibility of pleural amyloid and plasmocytic localisations devoid of pleural effusion. Another interesting point is that the disease at its onset mimicked RA, with symptoms common to AL amyloidosis and MM.

References