LETTERS

The autopsy revealed the following: 1 ) Chronic sial- oadenitis of the major salivary glands, showing lympho- plasmacytic infiltration associated with acinar atrophy and fatty infiltration. 2) Systemic necrotizing vasculitis charac- terized by (a) multiple, tiny, softened and hemorrhagic foci of the slightly edematous brain, which histologically re- vealed transmural and segmental necrotizing vasculitis with fibrinoid necrosis of the small arteries and arterioles in the subarachnoidal space (Figure 1) and cerebrum (deposition of immunoglobulin was minimal in the arterial walls); (b) necrotizing angiitis in other organs, especially in the gastro- intestinal tract, the kidneys, and the pancreas; and (c) focal glomerulonephritis with crescent formation. 3) Hemorrhagic bronchopneumonia of both lungs, associated with throm- bophlebitis of the vena cava inferior and left common iliac vein with bacterial colonies. 4) Perforating ulcers of the stomach and sigmoid colon.

From the clinical disease course and the autopsy findings, it is speculated that the complicating angiitis was mostly related to the underlying SS and could conceivably have been triggered by provoking factors such as adminis- tration of antibiotics or by bacterial infection. The immediate cause of death was sepsis with bronchopneumonia and perforation of the gastrointestinal ulcers (which could also be attributable to angiitis).

Alexander et al (2) reported that the presence of anti-SS-A (Ro) antibodies defines a subset of SS patients who have systemic manifestations including vasculitis, he- matologic abnormalities, and serologic hyperreactivity. Those researchers also recently claimed that 2 types of inflammatory vascular disease (IVD) occur in SS: neutro- philic IVD (NIVD) and mononuclear IVD (MIVD) (3). NIVD was significantly associated with serologic hyper- reactivity and with hypocomplementemia, whereas MIVD tended to occur in autoantibody-negative patients who had normal complement levels. Our patient had features that resembled MIVD. Steinberg et al(5) described 3 SS patients with thrombotic thrombocytopenic purpura. Although our patient did not initially have hemolytic anemia and fragmen- tation of red blood cells, her death might be explained by thrombotic thrombocytopenic purpura. In any event, sys- temic vasculitis has to be taken into consideration as one of the important factors determining the prognosis in patients with primary Sjogren’s syndrome.

1.

2.

Kazuto Sato, MD Nobuyuki Miyasaka, MD Kusuki Nishioka, MD Tokyo Women’s Medical College Kunio Yamaoka, MD Masaharu Okuda, MD Takaaki Nishido, MD Hisataka Uchima, MD Tokyo Medical and Dental University Tokyo, Japan

Strand V, Talal N: Advances in the diagnosis and concept of Sjogren’s syndrome (autoimmune exocrinopathy). Bull Rheum Dis 30: 104fj-1052, 1980 Alexander EL, Provost TT, Arnett FC, Stevens MB: Sjogren’s syndrome: association of anti-Ro (SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyper- reactivity. Ann Intern Med 98: 155-159, 1983

3. Molina R, Provost TT, Alexander EL: Two types of inflamma- tory vascular disease in Sjogren’s syndrome: differential associ- ation with seroreactivity to rheumatoid factor and antibodies to Ro (SS-A) with hypocomplernentemia. Arthritis Rheum 28:

4. Kaltreider HB, Talal N: The neuropathy of Sjogren’s syndrome: trigeminal nerve involvement. Ann Intern Med 70:751-762, 1969

5 . Steinberg AD, Green WT, Talal N: Thrombotic thrombocyto- penic purpura complicating Sjogren’s syndrome. JAMA 215: 757-761, 1971

1251-1258, 1985

Genetics of the Hajdu-Cheney syndrome

To the Editor: In their case report and literature review of idio-

pathic familial acroosteolysis (Hajdu-Cheney syndrome), Udell and colleagues seem to assign some importance to the observation that “. . .in each of the 2 families that have been HLA typed, both mother and son share 1 similar locus. . .” (1). This observation, whether applied to their patient, who had the familial form of the disorder, or to Elias’ patient, who had the nonfamilial form (2), is at best a truism.

Because the genes of the HLA system are inherited in classic Mendelian fashion, it is axiomatic that children will have a haplotype in common with each of their biologic parents. Of interest, and of possibly greater importance, then, would be knowledge of the HLA typing results in unaffected siblings of the proband.

Although the disease is classified as a genetic disor- der with presumed autosomal dominant mode of inheritance (3), the gene(s) responsible for development of Hajdu- Cheney syndrome has not yet been mapped or assigned to a locus on any human chromosome. The rarity of the disorder, the occurrence of both familial and nonfamilial forms, and the absence of associated immunologic abnormalities make it unlikely that gene products of the human major histo- compatibility complex are significantly involved in the pathogenesis of this osteolytic condition. To date, the re- ports, including Udell’s, neither confirm nor refute this speculation.

Allan Gibofsky, MD, JD, FACP The Hospital for Special Surgery New York. NY

1. Udell J , Schumacher HR Jr, Kaplan F , Fallon MD: Idiopathic familial acroosteolysis: histomorphometric study of bone and literature review of the Hajdu-Cheney syndrome. Arthritis Rheum 29:1032-1038, 1986

2. Elias AN, Pinals RS, Anderson HC, Gould LV, Streeten DHP: Hereditary osteodysplasia with acro-osteolysis (the Hajdu- Cheney syndrome). Am J Med 65:627436, 1978

3. McKusick VA: Mendelian Inheritance in Man. Baltimore, Johns Hopkins University Press, 1971, p 10

Effect of vitamin C dietary supplementation on survival in amyloidosis

To the Editor: Systemic reactive (AA) amyloidosis is usually a fatal

complication of otherwise nonfatal chronic infections and inflammatory conditions, and no effective therapy is known