The clinical findings and genetic bases of inherited deficiencies of plasma complement components and complement control proteins are reviewed. In Japan, since the frequencies of late complement component deficiencies (LCCD) are high, clinical features of neisserial infections associated with LCCD are described in details. C9 deficiency is one of the most frequent genetic disorders in Japan and most of them are healthy. However, C9 deficiency is weakly but significantly associated with the development of meningococcal meningitis but not of systemic lupus erythematosus. The common Arg 95 Stop mutation was found in most individuals with C9 deficiency. Molecular epidemiologic study revealed that homozygous and heterozygous Arg 95 Stop mutation of C9 gene is found in approximately one of 1000 individuals and one of 15 individuals, respectively. Complement studies including C9 antigen and DNA analyses should be performed in patients with meningococcal meningitis or recurrent bacterial infections.

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To measure the amount of erythrocyte-associated IgG (EAIgG) molecules from 70 patients with systemic lupus erythematosus (SLE) who had negative direct antiglobulin test (DAT), we employed a sensitive enzyme-linked immunosorbent assay (ELISA) technique. Ninety-five percent of healthy individuals were less than 65 molecules of EAIgG per red cell (RBC). About 51 percent of these patients with SLE were positive (over 65 molecules of EAIgG per RBC) by this technique that showed more sensitive than the DAT. EAIgG levels of these patients were inversely proportional to RBC count (r=-0.369, p<0.005), and EAIgG positive patients were recognized by 88 percent in patients group who were less than 4×10⁶/μl RBC counts. These results suggest that even a few EAIgG, as of a DAT shows negative, are related to the catabolism of erythrocytes in patients with SLE.
EAIgG determination value in this method calculated and converted with CRM 470 that is the IFCC international reference preparation for plasma protein.

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Objective: The most important diagnostic criterion in Sjögren's syndrome (SS) is considered to be the histologic focus score of the labial salivary glands. The focus score is defined as the number of lymphocytic foci per 4mm² of the salivary gland according to the criterion of Chisholm & Mason. On the other hand, in the criteria of the Sjögren's Disease Research Committee of the Ministry of Health and Welfare in Japan it is defined as the number of lymphocytic foci per a lobule of the salivary gland. By setting the limited criteria for SS on the basis of objective signs of both dry eyes and dry mouth, we compared the usefulness of these two diagnostic criteria for the diagnosis of SS in terms of the sensitivity, the specificity and laboratory data.
Methods: The biopsy of labial salivary glands was performed in 245 patients (230 females and 15 males, with a mean age of 54.9 years) who were suspected of SS in our hospital during the time between 1975 and 1996. Labial salivary glands were histologically assessed and the focus score was calculated according to the criterion of Chisholm & Mason and to that of the Sjögren's Disease Research Committee, respectively.
Results: The average area per a lobule of the salivary gland was 0.70mm². According to the limited criteria for SS, the Japanese histologic diagnostic criteria showed a higher specificity (93.3%) and a lower sensitivity (23.5%). The sensitivity of the criterion of Chisholm & Mason was 72.1%, and the specificity was 80.0%. The margin of the lobule was sometimes difficult to be identified because of the fatty change and fibrosis in some salivary glands.
Conclusions: By comparing the two different histologic criteria using our limited criteria, it was better to use the histologic criterion of Chisholm & Mason as a criterion for the diagnosis of SS than that of the Sjogren's Disease Research Committee of the Ministry of Health and Welfare in Japan in terms of the sensitivity, the specificity and laboratory data.

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We report on a 35-year-old Japanese woman with systemic lupus erythematosus (SLE) in whom homonymous hemianopsia developed. In August 1987, SLE was diagnosed. In November 1987, the patient was admitted to our hospital because of severe thrombotic thrombocytopenic purpura; however, clinical symptoms improved after treatment with vincristine. In 1992, severe lupus nephritis developed but improved after steroid therapy. In February 1994, diplopia developed owing to inflammatory invasion of the orbit by acute maxillary sinusitis. Humphrey Field Analyzer perimetry revealed a homonymous quadrantic hemianopic scotoma in the lower left side. Magnetic resonance imaging of the brain revealed a small infarct lesion in the posterior pole of the upper calcarine cortex. This lesion was thought to be responsible for homonymous hemianopsia. However, the lesion was not visualized with computed tomography. Our experience in the present case suggests that magnetic resonance imaging is more useful than computed tomography for identifying lesion causing visual field disorders in SLE.

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A 50-year-old female with systemic sclerosis (SSc) developed rapidly progressive renal insufficiency. Laboratory findings showed rapid elevation of serum creatinine level (3.8mg/dl) and a high titer of perinuclear-antineutrophil cytoplasmic antibody (p-ANCA) (504EU/ml). Renal pathology revealed crescentic glomerulonephritis (CrGN) without mucoid intimal proliferation of the interlobal arteries and fibrinoid necrosis of the afferent arterioles, Immunofluorescent micrography showed focal segmental granular deposition of IgG and C3 in the mesangium and along the capillary loop and was in agreement with pauci-immune type. Recently, a subtype of renal involvement in SSc that is associated exclusively with normotensive renal failure and recognizable by p-ANCA is suggested. On the other hand, SSc with p-ANCA-positive glomerulonephritis as this case can be considered to be overlap sydrome of SSc and microscopic polyangitis nodosa (microscopic PN) because in microscopic PN, p-ANCA is detected at the range of 50% to 80% and renal pathology reveals necrotizing glomerulonephritis. In this point, we may describe this case as a suggestive one about p-ANCA-positive glomerulonephritis.

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We report three cases of enteropathy associated with systemic sclerosis that were improved with oral type II collagen. The first patient, who was diagnosed as having systemic sclerosis-polymyositis overlap syndrome at the age of 35, was hospitalized for treatment of renal crisis at the age of 36. He acquired severe enteropathy associated with systemic sclerosis. The renal crisis was improved with angiotensin-converting enzyme inhibitor, but enteropathy persisted. He was given type II collagen in order to treat ileus. Four weeks later, ileus was improved and he was able to take foods. The second patient, a 63-year-old female whose onset of systemic sclerosis was at the age of 58, complained of ileus several times and was given oral type II collagen. Six weeks later, ileus was improved. The third patient, a 19-year-old female, was diagnosed as having systemic sclerosis-polymyositis overlap syndrome. Enteropathy was so severe that she could not take foods for 1 year. She was given oral type II collagen. Six months later, ileus and skin sclerosis were markedly improved. These results suggest that oral type II collagen is effective in the treatment of gastrointestinal dysfunction associated with systemic sclerosis.

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