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The pond snail Lymnaea stagnalis can often be observed moving upside down on its back just below the surface of the water. We have termed this form of movement "upside-down gliding." To elucidate the mechanism of this locomotion, we performed a series of experiments involving behavioral analyses and microscopic observations. These experiments were designed (1) to measure the speed of this locomotion; (2) to determine whether the mucus secreted from the foot of Lymnaea repels water, thereby allowing the snail to exploit the surface tension of the water for upside-down gliding; and (3) to observe the beating of foot cilia in this behavior. The beating of these cilia is thought to be the primary driving force for upside-down gliding. Our results demonstrate that upside-down gliding is an efficient active process involving the secretion of mucus that floats up to the water surface to serve as a substrate upon which cilia beat to cause locomotion at the underside of the water surface.

Objective: Clinical pictures of poly-myalgia rheumatica (PMR) and remitting seronegative symmetrical synovitis with pitting edema (RS3PE) are often indistinguishable from those of early rheumatoid arthritis (RA). To investigate whether there is a difference in immunological aspects among these 3 disorders, we performed a phenotypic analysis of peripheral blood lymphocytes., Patients and Methods: Eleven patients with early RA, 14 with PMR and 11 with RS3PE were enrolled in this study. After separation of mononuclear cells from peripheral blood using the Ficoll-Hypaque method, surface markers and intracellular cytokines of lymphocytes were analyzed by 2- or 3-color flow cytometry., Results: Both PMR and RS3PE showed a significant decrease in CD8 + CD25 + cells (p<0.05), and significant increases in CD4 + IFN-gamma + IL-4- (p<0.05), CD8 + IFN-gamma + IL-4- (p<0.05 and p<0.01, respectively) and CD4 + TNF-alpha + cells (p<0.05) compared with early RA. CD3 + CD4 + cells were higher in PMR than in RS3PE (p<0.01), but there were no significant differences in any other phenotypes between these disorders., Conclusion: A decrease in activated cytotoxic/suppressor T cells and increases in circulating Th1 and Tc1 cells may be common characteristics of PMR and RS3PE in comparison with early RA. Both disorders are clearly different from early RA, and probably belong to the same disease entity with regard to phenotypes of peripheral blood lymphocytes.

We report 3 patients with nephrotic syndrome ascribed to primary systemic AL amyloidosis that were successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone. M-protein in serum disappeared soon after VAD, and nephrotic syndrome gradually improved in parallel with a decrease in daily protein excretion in urine. Long-term follow-up of these patients showed neither relapse of nephrotic syndrome nor reappearance of M-protein. High-dose melphalan followed by autologous stem cell support is a standard therapy for primary systemic AL amyloidosis, but in high-risk cases for this treatment, such as elderly patients and those with multiple organ involvement, VAD might be a therapeutic option.

Objective: Intensive chemotherapy targeting plasma cell dyscrasia has been recently employed for the treatment of primary systemic AL amyloidosis. We prospectively studied the clinical usefulness of cyclic VAD (vincristine, doxorubicin and dexamethasone) in patients with primary systemic AL amyloidosis who were ineligible for high-dose melphalan with autologous stem cell support., Patients and Methods: Eight patients (mean age, 60.4+/-8.8 years) were treated with cyclic VAD until the disappearance of M-protein from both serum and urine. Of these, seven showed nephrotic syndrome before the start of VAD irrespective of a decrease in creatinine clearance. Serial follow-up studies after VAD evaluated hematological status and organ function., Results: Four patients (50%) showed a marked decrease in abnormal plasma cells in the bone marrow and normalized kappa/lambda ratios of serum free light chain in conjunction with disappearance of M-protein after 1 to 3 courses of VAD. There were no serious adverse events, and nephrotic syndrome gradually improved with no hematological relapse in the follow-up period of 3 to 5 years. The remaining 4 patients showed worsening of congestive heart failure and/or systemic edema ascribable to dexamethasone, resulting in cessation of cyclic VAD before disappearance of M-protein. All of these patients died of multiple organ failure or required permanent hemodialysis within 1 year after the start of cyclic VAD., Conclusion: Cyclic VAD is a potent therapeutic option in primary systemic AL amyloidosis, but in patients with renal or cardiac dysfunction careful management for adverse events, especially body fluid retention, is necessary.

We report on a 54-year-old woman with rheumatoid arthritis who had severe femoral nerve palsy affected by a distended synovium in the hip joint. Surgical exploration demonstrated a perforation of the iliopectineal bursa connecting with the hip joint. The patient fully recovered from femoral nerve palsy after surgery. It was considered that synovitis of the hip joint had developed following huge iliopectineal bursitis.

We report a patient with myeloma-associated systemic AL amyloidosis who showed chronic polyarthralgia as the main symptom. The clinical picture was similar to that of rheumatoid arthritis with regard to symmetrical swelling with tenderness in multiple joints, but inflammatory reactions were almost normal and autoantibodies were negative. He was diagnosed as having systemic AL amyloidosis based on deposition of kappa-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in serum and urine. Magnetic resonance imaging and bone scintigraphy suggested this disease as the cause of the polyarthralgia. Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia.

Objective: Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies; autoimmune mechanisms are thought to play an important role in their pathogenesis. We investigated the immunocytochemical characteristics and Th1/Th2 balance of peripheral blood lymphocytes in PM and DM using flow cytometry., Patients and Methods: Eight patients with PM and 13 with DM were enrolled in this study. Of these, 8 patients with DM were examined before and after clinical remission. No patients were receiving any treatment for PM or DM at enrollment. Ten healthy subjects were used as controls., Results: Patients with PM showed significant increases in CD3+CD4+HLA-DR+ (p<0.01) and CD19+CD23+ cells (p<0.05), and significant decreases in CD3+CD4+ (p<0.005) and CD4+CD45RO+ cells (p<0.05) compared with controls. Patients with DM showed significant increases in CD19+ (p<0.05) and CD19+CD23+ cells (p<0.05), and significant decreases in CD4+CD45RO+ cells (p<0.005) and the CD4+CD45RO+/CD4+CD45RA+ ratio (p<0.005) compared with controls. CD4+interferon (IFN)-gamma+ cells and the intracellular IFN-gamma/interleukin (IL)-4 ratio in CD4+ cells were significantly lower in patients with DM than in those with PM (p<0.05) or controls (p<0.0005 and p<0.001, respectively). The intracellular IFN-gamma/IL-4 ratio in CD4+ cells was significantly increased in DM after clinical remission compared with before (p<0.05)., Conclusion: Both B and helper T cells are activated in peripheral blood of active PM. Th2 cells predominate in peripheral blood of active DM, and the intracellular IFN-gamma/IL-4 ratio in CD4+ cells may be useful as a clinical marker indicating disease activity.

Objective: Primary systemic AL amyloidosis arises from immunoglobulin light chains produced by plasma cell dyscrasia. To prospectively investigate the production of M-protein and plasma cells in bone marrow before and after chemotherapy, we performed flow cytometry and analysis of serum free light chains (FLCs)., Patients and Methods: Fifty-nine patients with primary systemic AL amyloidosis (mean age, 59.9+/-8.8 years) were enrolled in this study, and of these 31 were serially studied before and after chemotherapy. Complete hematological remission was defined as normalization of the FLC kappa/lambda ratio., Results: MPC-1(-)CD45(-) (p<0.05) and MPC-1(+)CD45(-)CD49e(-) (p<0.005) were significantly higher, and MPC-1(-)-CD45(+) (p<0.05), MPC-1(+)CD45(+)CD49e(-) (p<0.0001) and MPC-1(+)CD45(+)CD49e(+) (p<0.0005) were significantly lower in the patients with AL amyloidosis than in controls. There was a significantly positive correlation between the serum predominant FLC/serum creatinine ratio and MPC-1(+)CD45(-)CD49e(-) (p<0.05). After chemotherapies, such as high-dose melphalan with autologous stem cell support, 20 of 31 patients with AL amyloidosis achieved complete hematological remission. There were no significant differences in any subtype of plasma cells before treatment between the remission and non-remission groups, but in the former group MPC-1(+)CD45(-)CD49e(-) and MPC-1(-)CD45(+) were significantly decreased and increased after chemotherapy compared with before, respectively., Conclusion: Abnormal plasma cells in the bone marrow, particularly the MPC-1(+)CD45(-)CD49e(-) subset, may be important as a follow-up marker before and after chemotherapy in primary systemic AL amyloidosis. These cells maintain low levels as long as no relapse occurs.

Interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ADM) is a rare and sometimes fatal condition whose clinical features are not well understood. The goal of this study was to clarify the characteristics of ILD based on its development. Eighteen patients diagnosed with ILD associated with ADM were assigned to 1 of 2 groups: (1) a rapidly progressing group, which included patients who developed abnormal lung findings within 1 month of being diagnosed with ADM (n=9); or (2) a slowly progressing group, which including patients who developed lung findings greater than 1 month after diagnosis of ADM (n=9). Serum creatine phosphokinase and C-reactive protein levels were higher in the rapidly progressing group than in the slowly progressing group. Further, arterial pH was higher and PaO(2)/F(I)O(2) was lower in the rapidly progressing group than in the slowly progressing group. On thoracic high-resolution CT, traction bronchiectasis was present in 4 of the 9 rapidly progressing patients but not in any patients of the slowly progressing group. All 9 slowly progressing patients survived with proper treatment, but only 4 of the 9 rapidly progressing patients survived. In ADM, appropriate investigations are likely required for the early diagnosis of ILD. Our data suggest that ILD associated with ADM can be classified into 2 clinical subtypes based on the time course of pulmonary involvement. Patients with rapid progression in respiratory symptoms should undergo intensive treatment as soon as possible to promote favorable outcomes.

Objective: Tristetraprolin (TTP), T cell intracellular antigen 1 (TIA-1), and Hu antigen R (HuR) are adenine/uridine-rich element binding proteins (ABPs) that affect the production of tumor necrosis factor alpha (TNFalpha) by binding to TNF messenger RNA (mRNA). TTP promotes deadenylation, TIA-1 inhibits translation, and HuR stabilizes TNFalpha mRNA. The aims of this study were to understand the posttranscriptional control of TNFalpha production in patients with rheumatoid arthritis (RA), and to identify parameters that may predict the efficacy of anti-TNFalpha therapy., Methods: Peripheral blood mononuclear cells from 38 patients with RA were obtained before therapy and 2 weeks and 54 weeks after administration of the first dose of infliximab, and from 20 healthy control subjects. TNFalpha, TTP, TIA-1, and HuR gene expression levels were analyzed by real-time polymerase chain reaction., Results: At baseline, TTP and HuR gene expression levels, as well as the TTP:TNFalpha, TTP:HuR, and TIA-1:TNFalpha gene expression ratios were lower in patients with RA than in control subjects, while expression of TNFalpha, TIA-1, and TIA-1:HuR was higher in patients with RA. The TTP:HuR expression ratio decreased significantly after administration of infliximab. Positive correlations were observed between TNFalpha and TTP, TNFalpha and TIA-1, TIA-1 and HuR, and TNFalpha and HuR gene expression in both healthy control subjects and patients with RA. At baseline, the TIA-1:HuR ratio tended to be higher in patients who achieved 50% improvement according to the American College of Rheumatology criteria (ACR50) at week 54 than in those who did not achieve at least an ACR20 response., Conclusion: Differences in ABP gene expression may affect TNFalpha gene expression. A higher TIA-1:HuR expression ratio might correlate with the response to infliximab therapy.

We performed histological and immunohistochemical analyses of the removed thymuses from 20 elderly (onset age > 60 years) and 23 young (onset age < 40 years) patients with myasthenia gravis (MG) who tested positive for serum anti-acetylcholine receptor (AChR) antibodies, but who did not have associated thymoma. In the elderly group, nine (45%) patients had accumulations of lymphocytes, indicating an atrophied thymus with loss of the basic structure. The elderly MG patients with atrophied thymic tissues had higher titres of anti-AChR antibody (59.6+/-81.0 nmol/L) than those with adipose infiltration of the thymus alone (20.1+/-20.9 nmol/L). In immunohistochemical studies using image analysis, both young patients and elderly patients with atrophied thymic tissues were found to have significantly higher levels of CD20 than age-matched controls (p < 0.005). Atrophied thymic tissues, often seen immunohistochemically in young MG patients, may also be found in elderly patients, particularly in those with high titres of the anti-AChR antibody, even though adipose infiltration is marked in these patients.

A 54-year-old woman complaining of muscle weakness and weight loss was admitted to our hospital with suspected polymyositis. Muscle biopsy revealed Langhans-type giant cells and noncaseating granulomas. Therefore, sarcoid myositis was diagnosed. The patient was treated with prednisolone, and the symptoms improved gradually. Generally, sarcoidosis is identified clinically in patients with foggy vision or mediastinal lymphadenopathy, but muscular weakness may be an infrequently observed initial symptom. Sarcoidosis should be considered in the differential diagnosis of polymyositis.

We report a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who showed high titers of anti-sulfated glucuronyl paragloboside (SGPG) IgM antibody without M-protein in serum. The patient was resistant to corticosteroids and immunosuppressants, but after administration of rituximab, clinical symptoms improved and the patient remained in a stable state for approximately 10 months. Rituximab may be a potent therapeutic option for refractory cases of CIDP irrespective of detectable M-protein in either serum or urine.

We prospectively investigated therapeutic and adverse effects of tacrolimus in seven patients with myasthenia gravis (MG) who were resistant to conventional therapies or could not be treated with thymectomy because of complications. Within two months of initiation of tacrolimus all the patients subjectively showed improvement of clinical symptoms, while both the quantitative MG score for disease severity and MG-activities of daily living profile were significantly decreased (p<0.05) 3 and 6 months after commencement compared with before. Nine months after initiation and later, MG temporarily exacerbated in two patients with rapid tapering of oral prednisolone and one non-thymectomized one. This drug is useful in the treatment of refractory patients with MG irrespective of thymectomy, particularly in the early phase after commencement. When tacrolimus is additionally used for treatment of MG, preceding drugs, particularly corticosteroids such as oral prednisolone, should be carefully tapered if necessary in order to prevent clinical exacerbation.

Neuropsychiatric symptoms are often seen in patients with systemic lupus erythematosus (SLE). To investigate the relationship between involvement of the nervous system and clinical factors, including autoantibodies and the activity of SLE, we retrospectively reviewed 25 patients with neuropsychiatric SLE (NPSLE, mean age: 35.2+/-12.2 years). As controls 37 SLE patients without neuropsychiatric manifestations (mean age: 31.8+/-15.8 years) were employed in this study. At the onset no significant differences were seen in any clinical factors between the patients and the controls except for serum antinuclear antibodies. In relapse, the patients with NPSLE showed significantly lower levels of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores without neuropsychiatric evaluation (p<0.0001), erythrocyte sedimentation rate (ESR, p<0.005), and antinuclear and anti-double-stranded DNA antibodies (p<0.005) and higher WBC values (p<0.05) than at the onset. Also in the patients with relapsing NPSLE similar significant differences were seen in these parameters between onset and relapse (p<0.005). Despite a lack of significant differences, the cerebrospinal fluid showed lower values in cell counts, total protein, and IgG in relapse than at onset. These results suggest that there are no clinical factors that predict the development of NPSLE and that relapse can occur with low disease activity in the nervous system even with an inactive state of other organ involvement. Since NPSLE may suddenly relapse with a slight change in common disease activation markers, including inflammatory reactions, autoantibodies, and complements in serum and CSF findings, adequate corticosteroid and/or immunosuppressive agents should be given at the onset and gradually be tapered after recovery, while looking out for recurrence.

We report a patient with polymyalgia rheumatica (PMR) who showed a relapse soon after tapering of oral prednisolone. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were quickly normalized after the re-increase in oral prednisolone, and muscle pain and stiffness gradually improved in parallel with a decrease in serum amyloid A (SAA). Flow cytometry simultaneously demonstrated an increase in CD8+CD25+ cells and a decrease in CD4+CD25+ cells and CD4+CD45RA+ cells. When clinical symptoms remain with negative results for CRP and ESR even after the start of corticosteroid treatment, SAA might be a potent therapeutic marker for disease activity in PMR.

A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart. In this patient natriuretic peptides and free light chains in serum were useful as markers of cardiac involvement and therapeutic effects, respectively. When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.

Objective: Immunoglobulin-related free light chains (FLCs) in serum have recently become quantitatively detectable using the nephelometric assay in plasma cell disorders, including multiple myeloma and AL amyloidosis. To investigate whether FLCs are useful as a diagnostic and therapeutic marker in Japanese patients with primary systemic AL amyloidosis, we determined these values in serum before and after chemotherapy., Patients and Methods: The serum FLC analysis was carried out in 25 patients with primary systemic AL amyloidosis (mean age, 60.1+/-8.4 years). All of the patients were shown to have either ALkappa- or ALlambda-immunoreactive amyloid deposits on biopsied tissues. Thirteen patients were treated with VAD (vincristine, doxorubicin and dexamethasone) alone (n=6) or VAD and subsequent high-dose melphalan followed by autologous stem cell support (n=7), and serum FLCs were serially determined before and after the chemotherapy., Results: Before chemotherapy the amyloidogenic FLC was elevated in serum with or without abnormal e/e ratios in 24 patients, including 5 with undetectable M-protein in both serum and urine on immunofixation. After chemotherapy the amyloidogenic FLC in serum was significantly decreased irrespective of high-dose melphalan (p<0.05), and all the patients with normalized kappa/lambda ratios showed a good prognosis., Conclusions: With respect to sensitivity and quantification serum FLCs will be a key marker for diagnosis and therapeutic effects in primary systemic AL amyloidosis. The prognosis of patients with this disease may be improved if the kappa/lambda ratio in serum can be normalized by intensive chemotherapy.

This report concerns two patients with severe protein losing enteropathy and refractory diarrhea due to AA amyloidosis who were successfully treated with corticosteroid and octreotide. In these patients, biopsied tissues from the gastrointestinal (GI) tract showed extensive deposition of AA amyloid, which was caused by rheumatoid arthritis in one case and was of unidentified etiology in the other. Both patients manifested severe diarrhea unresponsive to conventional treatment with hypoproteinemia, and protein leakage from the small intestine to the ascending colon was confirmed by 99mTc-diethylene triamine pentaacetic acid human serum albumin (HSA-D) scintigraphy. Soon after starting a long-acting somatostatin analogue, octreotide, with co-administration of oral prednisolone, their general status improved in parallel with a rapid decrease in the volume of watery diarrhea and an increase in serum levels of albumin and IgG. Also on 99mTc-HSA-D scintigraphy protein leakage from the GI tract was apparently decreased in both patients. Combination therapy with a somatostatin analogue and corticosteroid may be effective for protein losing enteropathy with intractable diarrhea ascribable to GI amyloidosis. Because of the lack of specific therapies in this serious clinical situation, the described therapy should actively be considered as a therapeutic option not only in AA amyloidosis, but also in other types of systemic amyloidosis.

To investigate whether there is a correlation between subtypes of plasma cells in the bone marrow and the production of M-protein, flow cytometry and serum free light chain (FLC) analyses were carried out in 17 patients with primary systemic AL amyloidosis (mean age, 59.9+/-8.8 years) and controls with M-protein (MGUS controls, n=6) and without it (negative controls, n=9). The patients showed a significantly higher value in the serum predominant FLC:serum creatinine ratio (43.8+/-63.2) and CD38++ CD19- CD56+ subpopulation (monoclonal plasma cells) (2.57+/-5.35%) than either the negative (p<0.0005 and p<0.001, respectively) or MGUS controls (p<0.05). With respect to maturation of plasma cells in the bone marrow, the intermediate (MPC-1+ CD45- CD49e-) and mature (MPC-1+ CD45+ CD49e-) subtypes were significantly higher (49.2+/-23.2%, p<0.005) and lower (27.6+/-21.3%, p<0.005) in the patients than in the negative controls, respectively. The serum predominant FLC:serum creatinine ratio was elevated in parallel with an increase in CD38++ CD19- CD56+ and MPC-1+ CD45- CD49e- cells and a decrease in mature subtypes (MPC-1+ CD45+ CD49e- and MPC-1+ CD45+ CD49e+ cells), There was a significantly positive correlation between the serum predominant FLC:serum creatinine ratio and either CD38++ CD19- CD56+ (r=0.510, p<0.05) or MPC-1+ CD45- CD49e- cells (r=0.481, p<0.05). In primary AL amyloidosis M-protein is probably produced by increased monoclonal plasma cells in the bone marrow, particularly by the intermediate subpopulation with a phenotype of MPC-1+ CD45- CD49e-.

Patients with AL amyloidosis were treated with VAD (vincristine, doxorubicin and dexamethasone) with or without high-dose melphalan followed by auto-PBSCT according to eligibility criteria based on disease severity, and prospectively investigated the therapeutic benefits and complications. Thirty-one patients were enrolled in this study. VAD and subsequent high-dose melphalan with auto-PBSCT were performed only in patients who met all of the eligibility criteria. Among patients ineligible for this treatment, VAD alone was performed in those with satisfactory general status. Eleven patients met the eligibility criteria, and of these, 7 were treated with VAD and subsequent high-dose melphalan with auto-PBSCT. Seven patients received VAD alone, and the remaining 17 were treated with the supportive therapy. Among the 14 patients treated with chemotherapy, 9 (5 of the 7 treated with VAD and high-dose melphalan, and 4 of the 7 treated with VAD alone) showed complete hematological response with apparent improvement of amyloidosis-related clinical symptoms. Serious complications of chemotherapy were cytomegalovirus infection and pneumocystis carinii pneumonia seen in 1 and 2 patients, respectively. These chemotherapies may be effective for reduction of M-protein and are also useful in improving of amyloidosis-induced organ dysfunction. In patients who cannot tolerate high-dose melphalan, VAD alone is a potent therapeutic option, although there are possible harmful effects on the heart and peripheral nerve.

We report on a carrier of human T-lymphotropic virus type 1 (HTLV-1) who developed mixed connective tissue disease (MCTD). This patient suddenly manifested clinical symptoms and interstitial pneumonia ascribable to MCTD following long-term infection with HTLV-1. After initiation of oral prednisolone all manifestations quickly improved in parallel with a decrease in inflammatory reactions. In this patient HTLV-1 infection might have played an important role in the pathogenesis of MCTD. Since HTLV-1 can cause adult T-cell leukemia and HTLV-1-associated myelopathy, and also collagen diseases including MCTD, careful observation is necessary even in a carrier, particularly when autoantibodies are detectable in serum.

This report concerns a patient with IgM AL amyloidosis due to a B cell lymphoproliferative disorder who was successfully treated with VAD and subsequent high-dose melphalan followed by autologous stem cell support. After this chemotherapeutic regimen, the patient showed complete hematological remission and improvement in nephrotic syndrome. These findings suggest that high-dose melphalan may also be effective for lymphoplasmacytoid cells producing monoclonal IgM which are phenotypically distinct from plasma cells. Myeloablative therapies, such as high-dose melphalan, should definitely be considered as a treatment option for AL amyloidosis, irrespective of the type of precursor immunoglobulin.

We report a patient with amyopathic dermatomyositis (DM) who mainly showed interstitial pneumonia and intractable skin necrosis in bilateral elbows and soles with a poor response to immunomediated therapy, including corticosteroid and high-dose intravenous immunoglobulin. Soon after starting oral cyclosporin A (CyA) the skin lesions healed completely and the interstitial pneumonia promptly improved in parallel with a decrease in serum KL-6. Since fatal interstitial pneumonia is frequently associated with amyopathic DM as in this case, administration of CyA should be actively considered as a therapeutic option when clinical symtoms are progressive and resistant to conventional treatments.

Examination of a 58-year-old woman who had developed severe upper back pain showed left peripheral type of facial nerve palsy, sensory disturbance of limbs and body trunk (Th7-9), cerebellar ataxia and generalized hyporeflexia. Upper back pain increased at night and was resistant to NSAIDs, antianxiety agents, opioids, and corticosteroids. Concentrations of serum CK and cerebrospinal fluid total protein were elevated. Electromyography (EMG) of perivertebral muscles (Th7-10) showed reduced recruitment and polyphasic potential. Several days later, our patient developed weakness of limbs and respiratory failure, and required mechanical ventilation. From these findings, we diagnosed her as having Guillain-Barré syndrome (GBS) and began treatment with intravenous immunoglobulin therapy. All symptoms including upper back pain improved gradually, and she had recovered almost completely by the 40th hospitalization day. Pain, especially lower back and leg pain, have attracted attention as common symptoms in GBS. However, only one GBS patient with initial severe upper back pain has been reported. Although the precise mechanism of pain in GBS remains unclear, EMG findings lead us to surmise our patient's pain originated from the nerve roots. Limb and back pain should therefore be taken into account for the diagnosis and treatment of GBS patients.

Objective: To detect amyloid deposits in the early phase of illness, we investigated the usefulness of abdominal fat aspiration biopsy along with genotyping of serum amyloid A (SAA) in patients with rheumatoid arthritis (RA)., Patients and Methods: We performed abdominal fat aspiration biopsy with phenol Congo red staining and determined inflammatory markers, including CRP and SAA, in 217 patients with an RA history longer than 5 years (mean age, 64.1 +/- 10.6 years). Genotypes of SAA1 and 2 were investigated in 127 patients with RA by a polymerase chain reaction-restriction fragment length polymorphism analysis., Results: In the abdominal fat aspiration biopsy 17 patients (7.8%) demonstrated amyloid deposits, which were confirmed as AA type by immunostaining on biopsied tissues from other organs, and nine of them showed no clinical symptoms ascribable to amyloidosis. RA patients with amyloidosis showed significantly higher serum levels of CRP (p < 0.05) and SAA (p < 0.0001) than those without amyloidosis. In the genotyping, amyloid deposition was significantly correlated with the frequency of SAA1.3 (p < 0.005 vs. 1.1, p < 0.05 vs. 1.5). Comparison of inflammatory markers between the number of SAA1.3 alleles showed that the SAA/CRP ratio and SAA concentration were higher in the 1.3 homozygote than in the others (p < 0.05). Two patients demonstrated amyloid deposits at the second abdominal fat biopsy one year after the first, and their SAA1 genotypes were 1.3/1.5 and 1.3/1.3., Conclusion: In RA patients confirmed as having SAA1.3, serial examinations with abdominal fat aspiration biopsy might contribute greatly to the early detection of amyloidosis during the long-term follow-up.

Objective: Systemic reactive AA amyloidosis is an intractable complication in patients with a long history of rheumatoid arthritis (RA). To help to more easily and reliably detect the presence of this form of amyloidosis in patients with RA and start intensive treatment as early as possible, we examined the sensitivity and usefulness of abdominal fat aspiration biopsy with phenol Congo red staining in the diagnosis of AA amyloidosis., Patients and Methods: Ten patients were diagnosed with systemic reactive AA amyloidosis secondary to RA (all women; mean age, 70.2 +/- 6.4 years; mean disease duration of RA, 20.3 +/- 11.2 years) based on histopathological examinations of biopsied specimens mainly from the gastroduodenal mucosa. Abdominal fat aspiration biopsy was performed in these patients, and the specimens were treated with both classical alkaline and phenol Congo red staining., Results: Phenol Congo red staining revealed amyloid deposits in all 10 patients, while conventional alkaline Congo red staining showed a positive result in 7 patients. In the patients with a positive result with alkaline Congo red staining, reactivity of one grade or two higher was demonstrated by the phenol Congo red method., Conclusion: Phenol Congo red staining is superior to the classical alkaline Congo red staining with respect to the detection of AA-amyloid deposits in biopsied abdominal fat tissue specimens. In addition to easy access and procedural safety, abdominal fat aspiration biopsy might contribute reliably to the diagnosis of systemic reactive AA amyloidosis secondary to RA when phenol Congo red staining is employed.