Sjögrens Syndrome Symptoms
- Dry Eyes
- Dry Mouth
- Musculoskeletal Manifestations
- Cutaneous Manifestations
- Pulmonary Manifestations
- Gastrointestinal Manifestations
- Renal Manifestations
- Endocrine Manifestations
- Hematologic Manifestations
- Central and Peripheral Nervous System Disease
- Constitutional Manifestations
Dry Eyes
Dry eye symptoms vary widely in severity. Lack of moisture renders the eyes more sensitive to both chemical and physical environmental irritants, producing a gritty sensation, conjunctival injection and, eventually, defects on the ocular epithelial surface. These symptoms worsen as the day progresses. Blepharitis, with which SS may be confused, is worse in the morning. Blurred vision, secondary to drying and disruption of the corneal epithelium, is a common complaint.
Dry Mouth
Dryness of the mouth is a cardinal manifestation of SS. The dryness frequently presents as difficulty swallowing (dysphagia), the inability to maintain a conversation without drinking fluids, the need to keep fluids at the bedside, alterations in the sense of taste and smell, problems in wearing dentures, and an increased frequency of dental caries. The oral mucosa is dry, and there is a diminished or absent sublingual salivary pool. Erythma of the mucosa may reflect oral candidiasis, which is frequently responsible for a generalized burning sensation in the mouth. The parotid and submandibular salivary glands may be enlarged. This is usually bilateral, and persistent asymmetry should raise the possibility of a neoplastic process.
Musculoskeletal Manifestations
Rheumatoid arthritis is the most common extra-glandular manifestation of SS. It has been estimated that 30-60% of Sjögrens patients have an arthritis with clinical and laboratory features indistinguishable from adult-onset rheumatoid arthritis. Generally, manifestations of arthritis appear to antedate the diagnosis of SS by 5-10 years. Systemic lupus erythematosus, progressive systemic sclerosis, and polymyositis may also be seen.
Cutaneous Manifestations
Cutaneous vasculitis may be seen in patients with SS, and its presence serves as a reminder of the systemic inflammatory nature of this disease. The most common lesions are palpable and nonpalpable pupuric lesions over the lower extremities. Urticaria-like lesions have also been observed. In contrast to classic urticaria, which is transient, these lesions persist for days and even weeks. Raynauds phenomenon, digital ulceration, erythema multiforme, erythema nodosum, and livedo reticularis may also be seen. Histopathologic study of vasculitic lesions has revealed a neutrophilic inflammatory process, indistinguishable from leukocytoclastic vasculitis, as the dominant finding. To a lesser extent, a mononuclear inflammatory process has been seen in which lymphocytes invade and destroy the integrity of the vessel wall.
Pulmonary Manifestations
Upper airway xerosis is frequently complicated by recurrent upper respiratory infections including sinusitis. Sjögrens patients may, less frequently, develop obstructive lung disease, and interstitial lung disease with impaired diffusion (DLCO). Bronchoalveolar lavage has revealed the frequent occurrence of alveolitis in SS.
Gastrointestinal Manifestations
Severe oral dryness may lead to dysphagia, with food "sticking" in the esophagus. This sensation is generally perceived in the upper portion of the throat. Increased fluid intake with meals to ease the passage of food is common. Reflux esophagitis, presumed due to the lowered amount of neutralizing saliva, may be seen. Paradoxically, chronic atrophic gastritis has also been reported, and there is a high incidence of anti-parietal cell antibodies, achlorhydria, and pernicious anemia. Approximately 10% of Sjögrens patients produce anti-mitochondrial and anti-smooth muscle antibodies. Mild elevations of hepatic enzymes may be seen. Furthermore, patients may demonstrate histopathologic liver abnormalities of primary biliary cirrhosis, chronic active hepatitis, and cryptogenic cirrhosis.
Renal Manifestations
Renal involvement in SS is most commonly in the form of distal tubular dysfunction causing renal tubular acidosis. Less frequently, more extensive tubular dysfunction may occur. Acute glomerulonephritis has been reported in SS, but is unusual. Acute interstitial nephritis due to medications may exacerbate the problem.
Endocrine Manifestations
Serum anti-thyroglobulin and anti-thyroid microsomal antibodies may occur in Sjögrens patients. Clinical evidence of Hashimotos thyroiditis has been detected in approximately 10-15% of patients. Pancreatic disease, of a subclinical nature, may be common in SS, but only rarely is it clinically relevant. Diabetes mellitus is no more common in SS than in the rest of the population.
Hematologic Manifestations
Anemia of chronic disease, hemolytic anemia, leukopenia, and thrombocytopenia may all be seen, and autoantibodies against all three hematopoietic cell lines have been detected. Paraproteins, including cryoglobulins, may be seen. Cryoglobulins in Sjögrens patients are often type II mixed cryoglobulins containing IgM-kappa monoclonal rheumatoid factor. Non-Hodgkins lymphomas, affecting particularly the cervical lymph nodes and salivary glands, occur with increased frequency in SS (relative risk 40-44). Sjögrens patients demonstrating extraglandular manifestations, including recurrent or persistent parotid swelling due to infiltration by non-neoplastic lymphocytes (pseudolymphoma), angioimmunoblastic lymphadenopathy, and splenomegaly, are at increased risk. Those patients who develop lymphomas frequently lose their autoantibodies and become hypogammaglobulinemic.
Central and Peripheral Nervous System Disease
Cerebrospinal fluid (CSF) analysis, histopathologic studies, psychiatric testing, neuroradiologic imaging, and electrophysiologic testing have emphasized that Sjögrens patients frequently exhibit neurologic manifestations involving the entire neural axis (i.e. brain, spinal cord, and peripheral nerves). Focal central nervous system disease, characterized by a small vessel vasculopathy, as well as more global CNS dysfunction, such as affective psychiatric disturbances, have been observed. Because symptoms may be multiple and vague, they may be considered "functional", and the patients hypochondriacal or neurotic. Formal cognitive function testing has revealed impairment of recent memory and concentration difficulties. Peripheral neuropathy has long been recognized in SS, and an isolated trigeminal neuropathy appears to be the most common presentation. Sensorineural deafness, mononeuritis multiplex, and small fiber sensory neuropathy may also be seen.
Constitutional Manifestations
Fatigue is commonly reported in SS. This may be a manifestation of the underlying inflammatory process, or be secondary to anemia, hypothyroidism, or an associated sleep disorder in the setting of depression, nocturnal polydipsia, or nocturnal myoclonus.