Extraglandular Sjogren’s – Thrombocytopenia

This lady presented to the hematologist with complaints of petechial rash. She was noted to be thrombocytopenic and was treated as a case of Idiopathic Thrombocytopenic Purpura. She was started with Prednisolone and Azathioprine.

During one of her follow up visits to the hematologist, she was noted to have a ANA of 1:640. She was then suspected to have SLE and the resultant thrombocytopenia. She was then referred to us.

ON reviewing her, she had no symptoms of SLE although she has dry eyes and dry mouth. Her anti dsDNA was negative. Her anti Ro and Anti La was positive. Her complement levels were normal. She has elevated globulin levels.

The provisional diagnosis is Sjogren’s thrombocytopenia.

J Assoc Physicians India. 2011 Feb;59:114-5.
Sjogren’s thrombocytopenia.
Kamath V, Prabhakar B, Veena, Lachikarathman D.
Department of Medicine, Bangalore Medical College and Research Institute, Bangalore, Karnataka.
Primary Sjogren’s is a multisystem autoimmune disease which predominantly affects the exocrine glands. pSS may occasionally present in an atypical way which may defy correct diagnosis for a considerable period of time. Clinically important immune-mediated cytopenia (or a combination of cytopenias) may be the first manifestation of an occult SS and have only been rarely described with Sjogren’s. This case exemplifies the atypical presentation of pSS and hence should be considered in the differential diagnosis of patients with unexplained cytopenias.

QJM. 2000 Dec;93(12):825-9.
Immune cytopenias as the presenting finding in primary Sjögren’s syndrome.
Schattner A, Friedman J, Klepfish A, Berrebi A.
Department of Medicine and. Hematology Unit, Kaplan Medical Center, Rehovot, and the Hebrew University-Hadassah Medical School, Jerusalem, Israel. amiMD@clait.org.il
A diagnostic delay of several years in primary Sjögren’s syndrome is common, even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report on three such patients who presented as Coomb’s positive haemolytic anaemia, systemic symptoms with agranulocytosis and gingival bleeding due to immune thrombocytopenia, to alert clinicians to the fact that primary Sjögren’s syndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. Sjögren’s syndrome, a common autoimmune disorder, should be considered in the differential diagnosis of apparently ‘idiopathic’ cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.

Nihon Rinsho Meneki Gakkai Kaishi. 2000 Apr;23(2):124-8.
Hematological abnormalities of primary Sjogren’s syndrome.
[Article in Japanese]
Aoki A, Ohno S, Ueda A, Ideguchi H, Ohkubo T, Hagiwara E, Shirai A, Oketani M, Nagaoka S, Senuma A, Ohota S, Matsunaga K, Ishigatsubo Y.
First Department of Internal Medicine, Yokohama City University School of Medicine.
Sjogren’s syndrome (SS) is an autoimmune disease characterized by a chronic inflammatory response mainly localized to the lacrimal and salivary glands. However, it sometimes involves extraglandular organs culminating in systemic disorders. Hematological abnormalities are not uncommon, although they rarely have clinical significance. In this study we examined 99 patients with primary SS who visited our hospital during 1989 to 1999. Patient’s mean age was 54.1 years and 95 out of 99 were female. Lymphopenia and leukopenia was noted in 35 patients (35.3%) and 26 patients (26.2%) respectively, and 7 patients (7.1%) had thrombocytopenia. 43 patients (43.4%) had either of these hematological abnormalities. Patients with lymphopenia showed significantly low frequency of arthralgia and anti-SS-A/B antibody was more common in this group. Only one patient in this group required prednisolone therapy because of polyarthritis and general fatigue while others needed no specific therapy. Patients with thrombocytopenia were significantly younger and a male/female ratio was higher than those without this abnormality. They had higher tendency to accompany with skin eruption, positive anti-SS-B antibody, anti-nuclear antibody and rheumatoid factor. Three out of 8 patients with thrombocytopenia were treated with prednisolone according to the protocol for idiopathic thrombocytopenic purpura. All of 3 patients had positive PA-IgG and normocellular bone marrow. Autoimmune mechanism such as polyclonal B cell activation may play a role in the pathogenesis of thrombocytopenia.

Interestingly the association of pseudothrombocytopenia in Sjogren’s syndrome:-

Nihon Rinsho Meneki Gakkai Kaishi. 1997 Apr;20(2):139-44.
A case of Sjögren’s syndrome associated with EDTA-dependent pseudothrombocytopenia.
[Article in Japanese]
Hosokawa T, Hinoda Y, Imai K.
Department of Internal Medicine, Ashibetsu Municipal Hospital.
A 69-year-old woman was admitted to Department of Orthopedic Surgery in our hospital because of lumbago on April 4, 1995. Since laboratory data showed thrombocytopenia (platelet count 2.1 x 10(4)/mm3) on admission, she was transferred to Department of Internal Medicine for further examination on April 11. She noticed abnormal taste and showed remarkable sicca symptoms. Schirmer test, gum test and electrogustometry were positive, and parotid sialogram findings and histology of minor salivary glands of the lip were compatible with those of typical Sjögren’s syndrome. Thus, she was diagnosed as Sjögren’s syndrome. Although the antibodies to SS-A/SS-B were negative in her serum, anti-nuclear and anti-centromere antibodies were strongly positive (x1280). Serum IgM level was increased. The decreased platelet count was observed when EDTA was used as anticoagulant. The binding activity of the anti-platelet antibody activated by EDTA was dependent on temperature. Its immunoglobulin class was shown to be IgM by enzyme-labelled antibody method. We here report a case of Sjögren’s syndrome associated with EDTA-dependent pseudothrombocytopenia.