Pulmonary sarcoidosis / antiphospholipid antibody syndrome

1. Case presentation:
A 41-year-old woman has pulmonary sarcoidosis
which was diagnosed in 2018. She was treated with
corticosteroids and immunosuppressants for years,
but the patient developed interstitial lung disease with
symptomatic signs (stage IV). Our patient mentioned
to have a two miscarriages.
She presented to the Internal Medicine consult with
unilateral swelling of the left upper limb, painless with
skin changes, the Doppler ultrasound revealed deep
Vein Thrombosis at left brachial and axillary veins and
she had unexpectedly complicated by abdominal pain
which CT scan showed mesenteric arteries
thrombosis,
A complete blood count was white cell count of 1200/
mm3 and hemoglobin of 9.5 g/dl, platelets count of
181,000/ mm3. hepatic and renal function tests,
electrolytes and calcium were all normal as were
immunoglobulin levels .An ECG showed sinus
tachycardia at a rate of 120, with an right axis
.Echocardiography demonstrated dilatation of the
right ventricle, severe pulmonary hypertension with a
pressure of 45 mmHg. Diffuse ILD and no pulmonary
embolus were detected in thoracic CT scan.
The patient was placed on low molecular weight
heparin (60 mg twice daily).
Laboratory studies showed that inherited
thrombophilia tests, Serum antinuclear antibody,
ANCA were all negative. Anti β2- glycoprotein
antibodies of IgG and IgM isotype were positive at
high titer, while Anticardiolipin antibody and lupus
anticoagulant were negative. 12 weeks later, anti β2-
glycoprotein antibody was still persistent and a
definite diagnosis of APS was made as the revised
Sapporo criteria were fulfilled.
She was treated by lifelong anticoagulant ,after
overlapping with a vitamin K antagonist
(Acenocoumarol 2mg /day) and maintaining INR
within 2 – 3 ,associated with optimal management of
sarcoidosis and its complications. There weren’t
reported further thrombotic events while on
anticoagulation and the patient remains on follow-up
consult.
Discussion:
Sarcoidosis is a multisystemic inflammatory disease
of unknown etiology characterized by histologic
presence of non caseating granulomas. The disease
follows a variable clinical course in various patients
[1].
Sarcoidosis is considered as autoimmune disorder
mediated primarily by CD4 T-helper cells and
mononuclear phagocytes and additionally Humoral
immunological abnormalities such as increased
immunoglobulins and autoantibodies including ANA
(29%), anti-ds DNA up to 6% and rheumatoid factor.
However, the overall clinical significance of these
antibodies is unclear at this time [2,4].
APLs have been reported to be present in various
autoimmune diseases ,Although they appear to be
fairly common in sarcoidosis, clinical features of
APS are apparently very rare. In a study involving 55
sarcoidosis patients by Y Ina et al,
APL antibodies of IgG and IgM isotype were detected
in 38% as compared with 7% of healthy controls ,they
considered the appearance of APL is not related to
disease activity of sarcoidosis but is an important
prognostic factor [5].
The APL antibodies occurred somewhat more
frequently in patients with more advanced stages of
sarcoidosis, a correlation was noted between the
occurrence of APL antibodies and pulmonary lesions
for periods longer than 2 and 5 years [1,5]. The
results in our case was 3 years which is consistent
with the previous studies.
Other case report suggests that APL antibodies may
contribute to organ damage in sarcoidosis[2]. Further
studies are needed to evaluate the prevalence of
clinical features of APS, particularly in patients with
advanced stages of sarcoidosis [2,3,4].
Conclusion:
Both antiphospholipid antibodies and antiphospholipid syndrome may occur in patients with sarcoidosis,
the association between sarcoidosis and APS was rarely reported, APL must be investigated when
appropriate clinical features such as pregnancy comorbidity and thrombosis.
la 4 ème journée de FMC DE L’AMIE &
LA SOCIÉTÉ ALGÉRIENNE D'OBÉSITÉ ET MALADIES MÉTABOLIQUES
Introduction:
Sarcoidosis is a chronic multisystemic non-caseating granulomatous disease of unknown cause of various
organs [1], Antiphospholipid syndrome (APS) is an autoimmune condition defined by persistently positive
APL as well as thrombosis events and pregnancy complications [2] , the association of sarcoidosis with the
APS is rare with few reported cases and not well defined. However, APL may be detected in sarcoidosis
generally without clinical consequences. We reported a case of well-documented pulmonary sarcoidosis
complicated by an APS.
Keywords: antiphospholipid (APL), Antiphospholipid syndrome (APS), sarcoidosis
References
1-Sarcoidosis and Antiphospholipid Syndrome: A Systematic Review of Cases Ranjan Pathak, Raju Khanal, Madan Raj Aryal, N Am J Med Sci. 2015 Sep; 7(9): 379–383.
2- Antiphospholipid syndrome in a patient with sarcoidosis: a case report R Singh, M D Conway, 2002 Research Article Find in PubMed
3- Antiphospholipid antibody syndrome in a patient with neurosarcoidosis Srinivasa D Satti, John Bartholomew, 1999 Research Article Find in PubMed
4- Antiphospholipid Syndrome Presenting As Massive Pulmonary Embolism in a Patient With Sarcoidosis Bahar Artım Esen , Esen Kıyan , EUR J GEN MED, 2005 -
5- Antiphospholipid antibodies. A prognostic factor in sarcoidosis Chest Ina Y, Takada K, Yamamoto M: 1994;105: 1179-83
Pulmonary sarcoidosis associated with antiphospholipid
antibody syndrome
D. Merdaci M.Benkaouha D.Azizi
Department of Internal Medicine kais hospital