Clujul Medical - Iuliu Haţieganu

Clujul Medical 2006 vol. LXXX - nr. 1
establised connective-tissue disease. The differential diagnosis included: systemic lupus
erythematosus, systemic sclerosis, rheumatoid arthritis, dermatomyositis and mixed
connective tissue disease. In order to better differentiate, it would have been useful to
measure separately, the titer of the anti-RNP and anti-Sm antibodies.
The treatment we initiated consisted of: corticotherapy (Metilprednisolon 32
mg/day), hepatotrophic therapy, antibiotic treatment (for the urinary tract infection),
local oral antimycotic and antihypertensive treatment (ACE inhibitor + beta blocker +
diuretic). After approximately 1 month of corticosteroid treatment the evolution was
excellent: remission of subjective clinical complaints, improved general status,
apyrexia, the disappearance of oral ulcerations and skin lesions, the regression of
hepatomegaly, the remission of ascites and pleurisy but the persistence of the pericardial
effusion. The humoral parameters improved ( ASAT = 121 U/l, ALAT = 68 U/l, CPK =
220 U/l, 24-hour urine proteins = 0,36 g/24 h, ANA = positive - 1/640).
The overall prognosis for UCTDs is better than that of other connective tissue
diseases. UCTD may remit permanently, progress to an established connective tissue
disease, or remain a stable chronic phase. In our case, the long term prognosis of the
patient is reserved due to the multi-organic dysfunction.
Discussion
The term “connective tissue disease” (CTD) includes a heterogeneous group of
conditions characterized by a wide variety of signs and symptoms. It refers to a group of
autoimmune disorders that are classified among the systemic rheumatic diseases and
include systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositisdermatomyositis
(PM-DM), primary Sjögren's syndrome (pSS), primary
antiphospholipid syndrome (APS), mixed connective tissue disease (MCTD), and
rheumatoid arthritis (RA). CTD share a number of epidemiological and immunological
features that suggest a common pathogenetic pathway [1]. Experimental data indicates
that genetic susceptibility to develop an autoimmune disease is multigenic and that
some genetic defects can predispose patients to more than one autoimmune disease [1].
The sharing of immunogenetic markers may lead to the development of common
clinical features. Among these, the most frequent are Raynaud's phenomenon and
arthralgia or arthritis, often associated with the presence of antinuclear antibodies and
rheumatoid factor. Taken together these features constitute a clinical syndrome that can
often represent the onset of a CTD. Nevertheless, it is known that patients presenting
with this syndrome cannot be diagnosed as having a definite CTD. The classification of
pauci-symptomatic conditions of this kind is still a matter of debate among
rheumatologists. To be diagnosed with one of the CTD, a patient must develop some
disease-specific manifestations, or alternatively a combination of nonspecific, but
characteristic findings. Since the majority of CTD-specific features are neither frequent
nor pathognomonic for a single disease, the most common way to identify CTD is to
look at a cluster of clinical and laboratory findings. This is also the major reason why
classification criteria have been developed for CTD [2]. They are referred to as
classification rather than diagnostic criteria [2, 3]. Classification and diagnostic criteria
certainly share a similar aim: to separate subjects with a definite CTD from those
without such CTD, including both patients with difficult to distinguish conditions and
healthy individuals [2]. The difference between them is that diagnostic criteria should
theoretically have 100% sensitivity and specificity while classification criteria may have
less. In conclusion, diagnostic criteria can be applied to every individual case, whereas
classification criteria cannot [2]. Classification criteria are selected by means of
statistical methods to cluster the combination of features to improve sensitivity and
specificity. It is worth noting that the higher the sensitivity and specificity of a
classification criteria set, the smaller the difference will be between diagnostic and
classification criteria. There also exists, however, a group of systemic autoimmune
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